Bilateral Edema Research Articles

Abstract 16992: Superior Vena Cava Syndrome in a Patient With Metastatic Primary Cardiac Diffuse Large B-cell Lymphoma - Case Report

Introduction: Primary cardiac tumors are uncommon, with an estimated incidence of 0.02%. Primary cardiac lymphomas (PCL), a subtype of non-Hodgkin lymphoma, account for about 10% of malignant and 1% of all primary cardiac tumors. Diffuse large B-cell lymphomas (DLBCLs) emerge as the predominant histological subtype within PCL cases. Case presentation: 54-year-old White male with no medical history presented with a few weeks of exertional dyspnea, nonproductive cough, and neck fullness. Exam was notable for tachycardia, jugular venous distension, bilateral upper extremity edema, and distended chest wall vasculature. Labs showed elevation of alkaline phosphatase and CRP. CT of the chest showed a right atrial mass extending into and obstructing the superior vena cava (SVC) and mediastinal lymphadenopathy (Panel A & B). Coronary CTA performed a few years earlier showed normal interatrial septum (Panel C) and coronary arteries. Transthoracic echo (TTE) confirmed a 7.6 x 5.0cm mass visualized in the interatrial septum extending to both atria. Cardiac MRI redemonstrated a large cardiac mass with malignant features suggestive of cardiac lymphoma vs angiosarcoma (Panel D-F). PET-CT showed enhanced fluorodeoxyglucose (FDG) uptake within cardiac mass, mediastinal, and intraabdominal lymph nodes (Panel G). Lymph node biopsy confirmed DLBCL. Patient underwent three cycles of EPOCH (etoposide phosphate, prednisone, vincristine sulfate, cyclophosphamide, doxorubicin hydrochloride) chemotherapy with excellent response. TTE following cycle 2 demonstrated decreased tumor size, and PET-CT after cycle 3 confirmed complete resolution of lymphadenopathy and minimal residual intracardiac FDG uptake. Conclusion: SVC syndrome can be a rare presentation of PCL. Multimodality imaging is important in cardiac mass characterization, diagnosis, treatment planning, and follow-up. Timely diagnosis and initiation of chemotherapy results in improved outcomes.

Abstract 12450: Mystifying Masquerade: Renal Cell Carcinoma Associated Arteriovenous Fistula Presenting as High-Output Heart Failure

Case Presentation: 59 year-old woman with CKD stage 3b and hypertension presented with 3 months of progressive dyspnea, abdominal distension, bilateral lower extremity edema, and a 60-pound weight gain. Physical exam noted diffuse anasarca, with stable hemodynamics on room air. EKG revealed low voltage leads and chest X-ray demonstrated an enlarged cardiac silhouette. CT abdomen was remarkable for significant ascites, body wall edema, and a suspicious large right renal mass. Diagnostic paracentesis indicated cardiac ascites. Echocardiogram showed preserved ejection fraction, moderate pericardial effusion and an enlarged right side of the heart. Right heart catheterization confirmed high-output heart failure (HOHF). A suspected renal mass-related arteriovenous fistula (AVF) was confirmed through inferior vena caval and renal venogram blood gas sampling, indicating arteriovenous shunting within the renal mass. Hepatic venogram excluded portal hypertension. MRI abdomen confirmed a large necrotic right renal mass consistent with renal cell carcinoma (RCC). Symptoms significantly improved after nephrectomy, with aggressive diuresis, and therapeutic paracenteses leading to an 80-pound weight loss. Postoperative echocardiography illustrated decrease in pericardial effusion and right ventricular size. Cardiac MRI and stress test were unremarkable prior to planned immunotherapy for stage III RCC. Discussion: AVF and HOHF associated with malignancy are unusual presentations. RCC is the most prevalent underlying malignancy linked with AVF and is hypothesized to be a result of hypervascularity caused by a VHL gene mutation. The most often utilized imaging modalities for detecting a tumor-related AVF are contrast enhanced CT or MRI. When imaging is inconclusive, a venogram with blood gas sample, as observed in our case, may be considered to establish an RCC-associated AVF.

Abstract 17328: A Case of Reversible Severe Systolic Cardiomyopathy; Thinking Outside the Box

Introduction: Non-ischemic systolic cardiomyopathy is associated with significant morbidity and mortality. Prompt evaluation and timely removal of the potential insult may lead to reverse remodeling and recovery of the left ventricular (LV) systolic function. Case: A 39-year-old female with BMI 40 presented with dyspnea and bilateral lower extremity edema. Vital signs significant for mild tachycardia and hypertension. ECG showed sinus tachycardia. Physical exam revealed bibasilar crackles and 3+ pitting edema of the lower extremities. Laboratory significant for NTpro BNP 563 pg/dL and TSH <0.005 IU/mL with a free T4 of 2.73 ng/dL. Chest x-ray showed pulmonary vascular congestion. Echocardiogram showed severely reduced LV systolic function with ejection fraction (EF) of 27%, global LV hypokinesis and diastolic LV diameter of 6.85 cm. No significant atrial or ventricular arrhythmias noted on Telemetry. A CT angiography of the coronary arteries showed no evidence of calcification or obstructive coronary disease. Additional labs revealed severely elevated TSH receptor antibody and thyroid stimulating immunoglobulin. Diffuse thyroid enlargement noted on ultrasound. Patient was diagnosed with Grave’s disease (GD) with Burch-Wartofsky score of 30 indicating an impending thyroid storm. She was treated with Metoprolol and Methimazole. She underwent intravenous diuresis and was started on guideline-directed medical therapy for systolic cardiomyopathy. In follow-up after 6 months, her TSH had normalized. On repeat imaging, her LVEF improved to > 50% with diastolic LV diameter of 5.6 cm. Discussion: This patient was diagnosed with non-ischemic dilated systolic cardiomyopathy due to severe hyperthyroidism from GD. Thyrotoxicosis-induced systolic cardiomyopathy occurs in about 1% of patients with GD and is associated with increased morbidity and mortality. Excess thyroid hormones in thyrotoxicosis can affect multiple regulatory and structural genes of cardiac myocytes resulting in abnormal intracellular calcium regulation and impaired LV systolic and diastolic function. Our experience is consistent with literature demonstrating the importance of timely diagnosis and treatment of this reversible cause of systolic cardiomyopathy.

Abstract 25 — Remitting Seronegative Symmetric Synovitis with Pitting Edema (RS3PE) in Dialysis Patient

Background Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome is characterized by acute symmetrical synovitis, and pitting edema involving, the dorsal part of hands and feet. It affects more men aged more than 50 years. The inflammatory markers are usually elevated, with negative rheumatoid factor. The underlying etiology is not clear but has been associated with drugs exposure, infections, malignancy in 30% of cases, and coexist with other autoimmune diseases. The clinical course is benign with significant improvement to low-dose prednisolone therapy for 2 to 3 months. It has been rarely reported in dialysis patients. Methods and case description A 54 years old male, known to have long standing uncontrolled diabetes mellitus, hypertension, chronic kidney disease and diabetic retinopathy. Recently, he was treated for left foot cellulitis and advanced renal failure (813 micromol/L) initiated on hemodialysis. He presented one week later with progressive painful bilateral upper limb and lower limb pitting edema, with limited range of movement in hands and reduce urine output. He was resumed on hemodialysis for management of volume overload. Despite removal of almost 12 liters of fluids during consecutive dialysis sessions and improvement of lower limb edema, his bilateral hand edema was persistence with pain and limited movement. Further investigations were done to evaluate for rheumatological condition. Results Hand X ray was normal and ultrasound of the hand revealed features of synovitis, diffuse subcutaneous oedema and possible cellulitis. He was started on IV antibiotics without major improvement and blood culture was negative. CT chest showed left pleural effusion. The ESR was elevated 120, while serology for HBV. HCV, HIV, and parvovirus were negative. In addition, the autoimmune workup were negative (CCP, RF, ANA, ANCA, C3, C4, ACEI). He was diagnosed with remitting seronegative symmetric synovitis with pitting edema (RS3PE). He was treated with IV hydrocortisone once 100 mg, followed by oral prednisolone 10 mg for 5 days with dramatic improvement in bilateral hand edema and movement. He had hyperglycemia steroid related managed with adjustment of insulin therapy. At follow up in 1 week, patient regain full function of both hands, and he was on regular hemodialysis trice per week. Conclusion RS3PE is rare in hemodialysis patients, with favorable response to steroid therapy. We used shorter duration of 5 days steroid therapy with complete recovery and no recurrence at 1 year follow up. Screening for underlying autoimmune, infection or malignancy in RS3PE is essential.

Using Compression Therapy to Treat Bilateral Lower Limb Edema in Patients with Lung Cancer: A Case Series.

Although rare, marked bilateral leg edema (BLE) can occur in patients with lung cancer. Systemic therapy for the underlying disease is important, but adjunct therapy might also be helpful. In this case series, the authors report on treating BLE in patients with lung cancer with compression therapy using elastic stockings and bandages. From April 2013 to March 2022, the authors conducted a retrospective survey of seven patients who developed marked BLE and received compression therapy. They evaluated effects based on improvements in subjective symptoms as well as objective findings 2 months after the start of the therapy. The bandage therapy was useful in patients who were driver-gene negative, but it was not effective in those who already had "progressive disease" with specific agents for their driver genes. No adverse events were observed. Compression therapy, even when attached or detached by nonmedical personnel, may provide favorable effects and should be considered as an adjunct treatment option in this population, in addition to effective systemic cancer therapy. These results indicate that a prospective clinical trial would be worthwhile.

Idiopathic bilateral occlusion of Foramen of Monro treated by septostomy with unilateral foraminoplasty: a rare case report

Introduction: Idiopathic occlusion of the Foramen of Monro is extremely rare in adults. The occlusion is classified into four types, with the first being the most infrequent. This condition induces noncommunicating hydrocephalus with the ensuing increased intracranial pressure symptoms. Headache is usually the chief complaint. Presentation of the case: The authors present a case of a 28-year-old female who presented with a chronic headache that was unresponsive to analgesics. No other neurological deficits were present. Fundoscopic examination revealed the presence of bilateral papillary edema. Computed tomography scan results showed bilateral enlargement of the lateral ventricles of the brain. A subsequent MRI scan ruled out secondary causes of occlusion, such as colloid cysts, meningiomas, or choroid plexus tumors, which entailed an idiopathic etiology. Treatment options include ventriculoperitoneal shunt insertion and septostomy with foraminoplasty. The former option is currently the treatment of choice, yet it is notorious for its ramifications, including foreign body reaction, breakage, and mechanical problems. The latter option is free of these risks; however, it requires meticulousness and precision to avoid damaging the fornix, which leads to impaired memory function. Conclusion: Septostomy with unilateral foraminoplasty could yield better outcomes if it is performed fastidiously.

Abstract 26 — Clinical Characteristics of Familial Mediterranean: Fever in Hemodialysis Patients

Introduction Familial Mediterranean fever (FMF) is an inherited genetic disorder characterized by recurrent episodes of fever, serositis, arthritis, and skin rash. Uncontrolled disease can lead to amyloid A disposition, nephrotic syndrome and progressive renal failure. Recurrence of AA amyloidosis post kidney transplantation has been reported. Long-term colchicine therapy will reduce the FMF attacks and risk of AA amyloidosis. Although there is high prevalence of FMF in MENA region, the data of end stage renal disease (ESRD) in FMF is limited. We report our center experience. Method A retrospective chart review study was conducted at Tawam Hospital over 12 years (January 2010 to January 2023). We included adult ESRD on hemodialysis with FMF disease and studied their management outcomes. Descriptive analysis was used. Results 1900 ESRD patients were on hemodialysis during study period, and only two patients were diagnosed with FMF and renal amyloidosis. Case 1: A 60-year-old male patient from Syria who is known to have FMF and polycystic kidney disease leading to ESRD. He underwent successful LRD kidney transplantation in 2009. He was on mycophenolic acid: 540 mg, BID, prednisolone: 5 mg, tacrolimus: 1 mg am, 0.5 mg and colchicine 0.5 mg BID. He developed progressive renal allograft dysfunction with evidence of nephrotic syndrome. The renal biopsy revealed recurrence of AA amyloidosis due to FMF in kidney allograft in 2018. He had decline in renal function over years was initiated on hemodialysis on 2022. He developed two attacks of FMF peritonitis while on hemodialysis that responded well to steroid/colchicine therapy. Case 2: 48 years old male, from Egypt, known to have irritable bowel syndrome. He presented to our hospital with bilateral lower limb edema, and progressive shortness of breath for one month. Investigations revealed heavy proteinuria (8.98 g/g Creat) and acute kidney injury. Serology and autoimmune workup were negative. Kidney biopsy showed renal amyloidosis, and chronic tubulointerstitial nephritis with severe interstitial fibrosis (60%). History taken again and was positive for FMF in his uncle. Genetic study and clinical confirmed the diagnosis of FMF. He was started on colchicine, but developed ESRD within 8 months and initiated on hemodialysis. He had attacks of FMF. Conclusion Renal amyloidosis in FMF patient leads to progressive renal failure despite colchicine therapy. In our dialysis cohort over 12 years, only two FMF patients were identified. Colchicine therapy is required in ESRD-FMF in order to managed FMF attacks and prevent other organ AA amyloidosis.

Papillary Renal Cell Carcinoma Type 1 Accompanied by Hemorrhagic Anemia: A Case Report

Background: Kidney cancer or renal cell carcinoma (RCC) accounts for 5% and 3% of all adult malignancies in men and women, respectively, thus representing the 7th most common cancer in men, and the 10th most common cancer in women. The incidence of kidney cancer in the last two decades has shown an increase, but in recent years the death rate due to kidney cancer has decreased because it is increasing.
 Case Presentation: A 27 year old male patient came with complaints of bloody urination. Patients also complain of body weakness. Every time they are active, the weakness decreases if the patient rests. The patient was stated to be suffering from anemia and a left kidney tumor. There was bilateral antebrachial pitting edema. There was bilateral pretibial edema. Cytological examination revealed papillary renal cell carcinoma type 1.
 Conclusion: Papillary cell carcinoma type 1 is a kidney tumor that has a good prognosis, especially if found at an early stage. The classic symptoms of type 1 pRCC are anemia, hematuria and kidney swelling.

B - 81 Neuropsychological Case Study: Transient Amnesia or "Sentinel Seizure?" Potential Prodromal Symptoms of an ACoA Aneurysm Rupture.

We present the neuropsychological profile of a 33-year-old female with a history of transient amnestic events preceding frontal intraparenchymal/intraventricular hemorrhage, bilateral frontal cerebral edema and subarachnoid hemorrhage following a rupture of an anterior communicating artery aneurysm (ACoA). Comprehensive neuropsychological evaluation referral by neurology to assist with treatment planning given cognitive and behavioral changes since rupture of ACoA aneurysm. Prior to hospitalization, transient episodes of feeling as if she had been "drugged at a party," followed by behavior changes (e.g., irritability/agitation, "not herself") were notable, while the patient was amnestic to the events; spanning days at a time. Intellectual functioning was well-preserved. Neuropsychological testing revealed weaknesses in visuospatial organization, word retrieval, and aspects of executive functioning. Most salient were deficiencies in memory; patient was amnestic to tests shortly after administration. Striking emotional lability/frequent bouts of laughing disproportionate to the conversation and social comportment inappropriate to the setting were present. To our knowledge, amnestic events preceding ACoA aneurysm rupture have not been reported, and perhaps the symptoms this patient experienced prior to the event were indeed prodromal in nature. Therefore, the potentially rare phenomena in the form of transient amnesia should be further investigated. While these symptoms of memory lapses may be nonspecific and unique to the patient, we also cannot rule out possible "sentinel seizure" as a potential warning sign.

The Relationship Between the Presence of Distal Collateral Lymphatic Flows and Obesity in Patients with Bilateral Lower Extremity Edema

Objective: This study aimed to comprehensively assess radionuclide imaging findings to aid in the clinical diagnosis of early-stage lower extremity lymphedema. Methods: Our retrospective study included 135 patients who underwent lymphoscintigraphy for bilateral lower extremity edema (BLEE) between 2017 and 2022. Of these patients, 117 (86.7%) were female. Lymphoscintigraphic images were acquired at the 10th, 40th, and 2nd hours post-injection of radiopharmaceuticals. Bilateral lower extremity lymphatic congestion (MLC) was observed in the 2nd-hour images of radionuclide imaging (RI), with an associated accumulation of activity in the inguinal lymph nodes. All patients presented with lower extremity edema. Results: Among our patients, 55 (40.7%) were classified as obese. Bilateral distal collateral flows (BDCF) were identified in 60 (44.4%) of our patients, all of whom were female. Within this group, 13 (21.7%) were obese, and 11 (18.3%) exhibited distal and direct muscle lymphatic congestion (DDMLC). DDMLC was present in 63 (46.7%) patients. Unilateral distal collateral flow (UDCF) was detected in 75 (55.6%) of the patients with BLEE, with 67 (89.3%) of them being obese. Obese patients exhibited a significantly higher prevalence of both UDCF and DDMLC (p: 0.0005). Detailed results can be found in Table 1-4 and Figures 3 and 4. Conclusion: Functional bilateral radionuclide imaging methods provide valuable data for physicians, enabling bilateral and comparative assessments. However, particular attention should be paid to the interpretation of asymmetric images, especially in specific patient groups.

SAT484 High Degree AV Block And Sinus Arrest: Cardiovascular Manifestations Of Myxedema Coma

Abstract Disclosure: J. Bosques-Lorenzo: None. P. Rivera: None. J. Colon-Castellano: None. Y. Otero-Dominguez: None. Bosques-Lorenzo Jaymilitte, Rivera-Cariño Patricia, Colon-Castellano Janet, Otero-Dominguez Yomaira. Background: Myxedema coma is a rare and life-threatening complication of severe hypothyroidism. The most common presenting symptoms are altered mentation, hypothermia, edema, and respiratory depression. The cardiovascular system is often involved, in the form of sick sinus syndrome, bradycardia, low voltage QRS complex, QT prolongation, and rarely cardiac arrest. Here we present a case of myxedema coma complicated with high degree AV block and sinus arrest. Clinical Case: A 84 year-old man with history of hypothyroidism on levothyroxine 125 mcg, presented to the ER due to disorientation, bilateral leg edema, and abdominal discomfort for 3 days. Vital signs revealed a temperature of 97.5 F, HR of 60bpm, BP 174/88 mmHg, and oxygen saturation of 100% at room air. Physical exam was pertinent for bilateral lower extremity edema, facial puffiness, macroglossia, slow mentation, and scattered rales on lung auscultation. Laboratories showed an elevated TSH (296.8 uIU/mL), with low total T3 and free T4 (45.03 ng/dL and 0.228 ng/dL respectively) with a myxedema score of 45. Altered renal parameters as per BUN and creatinine levels (83.4 mg/dL and 5.75 mg/dL) without electrolytes disturbances and elevated CPK (1250 U/L). Infectious process was ruled out. Chest X-ray showed vascular congestion. Echocardiogram showed a small pericardial effusion. Patient was admitted to ICU and started on IV Levothyroxine, along with hydrocortisone. Liothyronine was initially not available. On second admission day, the patient developed hypothermia (94.3 F) which was managed with passive warming. Telemetry monitoring recorded multiple episodes of bradycardia raging from 30 to 50 bpm, high-degree AV block, and one episode of sinus arrest of 15 seconds with self-resolution. Altered mentation persisted for which endotracheal intubation was performed to secure airway. A temporary transvenous pacing was placed emergently. Levothyroxine dose was further adjusted and liothyronine was initiated. After a few days of treatment, patient demonstrated clinical improvement that was supported with follow up thyroid function test. Patient was extubated and transitioned to oral levothyroxine. Nevertheless, he remained dependent of transvenous pacing for which permanent pacemaker placement was coordinated. Conclusion: Cardiovascular complications in myxedema coma are associated with poor prognosis with a mortality approaching 80%. Few cases of sinus or cardiac arrest associated to Myxedema coma have been reported in the literature. In this case, the patient developed high-degree AV block with concomitant sinus arrest despite prompt initiation of intravenous thyroid hormone. Prompt identification of cardiovascular involvement can prevent cardiovascular morbidity and mortality in myxedema coma. Presentation Date: Saturday, June 17, 2023

FRI552 Orbitopathy As The Sole Manifestation Of Hashimoto’s Thyroiditis In A Euthyroid Patient: A Case Report

Abstract Disclosure: C. Labib: None. Z. Saeed: None. M. Trabulsi: None. F. Ramharrack: None. INTRODUCTION: Thyroid associated ophthalmopathy (TAO) is caused by an autoimmune process involving the orbital tissue, however, its etiopathogenesis is still largely unclear. It is common in hyperthyroid patients with Graves’ disease and has also been reported in euthyroid and hypothyroid Graves’ patients with positive thyroid receptor antibodies (TRAb). However, only 5% of patient with Hashimoto’s thyroiditis (HT) develop orbitopathy. Here we report an unusual case of a male patient, clinically and biochemically euthyroid, who had severe bilateral exophthalmos due to underlying Hashimoto’s thyroiditis. CASE PRESENTATION: A 68-year-old male patient was evaluated for worsening bilateral eye protrusion associated with redness and grittiness for many years. The patient was otherwise asymptomatic; denied fatigue, any change in weight, constipation, joint/muscle pain or any other symptoms. On physical examination he was noted to have bilateral moderate periorbital edema, conjunctival injection along with mildly enlarged thyroid. Initial labs showed normal TSH 4.10 mIU/L (0.4-5.0mIU/L) and free T4 0.8 ng/dL (0.8-1.8 ng/dL). Further labs were notable for normal antithyrotropin antibody (TRAb) 1.09 IU/l and normal TSI less than 89%. Anti-thyroid peroxidase antibody (anti-TPO) level was as high as 12 IU/mL (less than 9 IU/mL). Thyroid US showed multinodular goiter with bilateral nodules. A Subsequent FNA was benign. The degree of exophthalmos on the Hertel ophthalmometer was 30 mm bilaterally. Orbital magnetic resonance imaging (MRI) showed proptosis of the left and right globes as well as prominence of intraorbital fat. Extraocular musculature was symmetric bilaterally and only minimally prominent but diffuse. Treatment with teprotumumab infusion was started with improvement in the patient’s symptoms. CASE DISCUSSION: Usually mild eye symptoms are seen in Hashimoto’s thyroiditis such as dryness, blurred vision, upper eyelid retraction and loss of lateral third of eyebrows. In rare cases it can cause orbitopathy similar to that seen in Graves’ disease. Like Graves’ orbitopathy, in addition to reversing the underlying thyroid abnormalities, moderate-to-severe orbitopathy is treated with corticosteroids or teprotumumab. This case sheds the light on exophthalmos being an atypical presentation of Hashimoto’s thyroiditis in a clinically and biochemically euthyroid patient. Physicians should be cognizant of this rare manifestation for prompt recognition and early treatment which can prevent corneal involvement and blindness due to optic nerve compression. Presentation: Friday, June 16, 2023

FRI572 Teprotumumab In Unexplored Presentation Of Thyroid Eye Disease

Abstract Disclosure: M.T. Torres Torres: None. A.N. Gonzalez Bossolo: None. M.M. Mangual Garcia: None. Thyroid eye disease (TED) is an autoimmune disease of the orbital soft tissue associated with facial disfigurement, diminished quality of life, and potentially vision-threatening consequences that can occur in 40% of patients with Graves’ disease (GD). All the therapies for this condition were off-label with questionable long-term effects until 2020 when the Federal Drug Administration approved teprotumumab, a human monoclonal antibody against insulin-like growth factor-1 receptor, for the treatment of TED. Randomized placebo-controlled trials have demonstrated that teprotumumab reduces proptosis, diplopia, and Clinical Activity Score (CAS) in patients with active TED of less than 1.3 years of duration who were treatment-naïve or previously treated with < 1g of methylprednisolone equivalent therapy. Data on the efficacy of teprotumumab on cases of longer TED duration or previously treated with eye surgery or > 1g of methylprednisolone equivalent therapy for TED is limited as these patients were excluded from the trials. A 76-year-old nonsmoker male with long-standing hyperthyroidism due to GD, glaucoma, and type 1 diabetes mellitus was referred by ophthalmologist due to TED. He received radioactive iodine (RAI) for treatment of GD after failure to Methimazole therapy. After RAI, he reported progressively worsening bilateral eye edema, redness, pain, vision loss, and diplopia requiring hospitalization for an emergent orbital decompressive surgery and intravenous steroid therapy due to sight-threatening Grave’s orbitopathy. He was discharged home with oral prednisone taper down until it was discontinued 6 months later with mild improvement of symptoms. Physical exam was remarkable for bilateral strabismus, orbital chemosis, conjunctival injection and redness, eyelid swelling, limited eye motion, severely impaired visual acuity and color vision, no proptosis, and a CAS of 5 suggesting active TED. Laboratories showed biochemical euthyroidism and controlled glycemia. Teprotumumab was started 2 years and 8 months after symptom onset with marked improvement of inflammatory signs and symptoms, eye motion, visual acuity, diplopia, intraocular pressure, and CAS score decrease to 1 point suggesting inactive disease within 4 weeks of therapy without adverse side effects. This case describes the positive effects of teprotumumab in a patient with characteristics not previously studied such as active TED of longer duration than in clinical trials, previous treatment with orbital surgery or > 1g of methylprednisolone equivalent therapy, and increased IOP. Rather than drawing conclusions, our goal is to create awareness and promote further research of teprotumumab and novel agents in patients with TED with previously uncharacterized presentations as it may have important implications for their management including significant improvement in their quality of life. Presentation: Friday, June 16, 2023

THU388 Insulin Edema Associated With Glargine: A Case Report And Brief Review Of Literature

Abstract Disclosure: J. Wood: None. V. Samji: None. F.S. Celi: None. P. Majety: None. Introduction: Insulin edema is an uncommon and poorly understood complication of insulin therapy in patients with diabetes. We report a patient with type 1 diabetes (T1DM) who developed edema within days of initiating insulin glargine that resolved with discontinuation of this medication. Case Report: A 20-year-old man with no past medical history presented with nausea, vomiting, polydipsia, and a 15-pound weight loss and was noted to have a hemoglobin A1c greater than 14.0%. Diabetic ketoacidosis (DKA) was ruled out and was started on weight-based insulin therapy with glargine and lispro three units with meals. Additional diabetes workup was notable for positive Anti-IA2 antibodies. Blood glucose levels improved prior to discharge. A week after discharge, he complained of swelling of limbs. Patient denied any shortness of breath, cough, chest pain, orthopnea, or nocturnal dyspnea. He was found to have extensive 2+ pitting edema in bilateral feet extending up to his calves. Dorsalis Pedis pulses were 2+ bilaterally. No warmth or erythema of legs was noted. His weight in clinic was 70.2 kg (154.4 lbs), up 11.6 kg (25.5 lbs) ten days post discharge. Evaluation for bilateral pedal edema was performed. Labs were notable for normal liver enzymes, creatinine, glomerular filtration rate and urine albumin levels, ruling out hepatic and renal causes of edema. Thyroid function tests were normal. His presentation was discussed with cardiology and brain natriuretic peptide level was drawn which was also normal. No further cardiac workup was recommended at that time given the patient’s history and lack of other clinical signs or symptoms of heart failure. After ruling out other systemic causes of edema in our patient, a diagnosis of insulin edema was made. Based on prior case reports of glargine associated insulin edema, he was switched from insulin glargine to insulin degludec. He noted significant improvement within 24 hours of switching insulin with a 6.4-pound weight loss in just one day and complete resolution of edema within 3 days. He had no recurrence of edema once glargine was discontinued despite increasing insulin dosing for glycemic control. Conclusion: The pathophysiology of insulin edema is unclear. Insulin’s role in renal sodium handling, vasodilation, and increased vascular permeability, have been postulated as possible mechanisms. Risk factors for developing insulin edema include T1DM, high A1C, high dose of insulin, rapid correction of hyperglycemia, DKA on presentation and poor nutritional status. Additional risk factors for developing insulin edema include thiamine deficiency and an underlying genetic predisposition related to a mitochondrial mutation, although this seems to be rare. Insulin edema is a diagnosis of exclusion. Clinicians should be aware of this rare complication, and its occurrence should be documented and differentiated from other causes of edema. Presentation: Thursday, June 15, 2023

FRI204 A Rare Case Of Cushing Syndrome In Pregnancy: Diagnosis And Therapeutic Challenges

Abstract Disclosure: Z. Cardona: None. E.D. Szmuilowicz: None. Introduction: Cushing’s syndrome (CS) rarely occurs during pregnancy, and diagnosis of Cushing’s syndrome in pregnancy is challenging due to physiologic changes during gestation. CS is associated with severe maternal and fetal complications. Clinical Case: A 36-year-old woman presented at 21 weeks gestational age with a known adrenal nodule admitted for evaluation of HTN, bilateral lower extremity edema, transaminitis, hyperglycemia, and acute hypoxemic respiratory failure. Reported proximal muscle weakness, facial swelling, anxiety, depression, perspiration, and headaches associated with HTN. Physical exam notable for mild supraclavicular fat deposition, chest and neck plethora, hypertrichosis, and bruising. CT abdomen pelvis revealed a 2.3-cm left adrenal nodule measuring 30 Hounsfield units. Biochemical evaluation included a plasma renin activity of 0.719 ng/mL/h [ref range: 0.167-5.380 ng/mL/h], aldosterone <1.0 ng/dL [ref range: 0.167-5.380 ng/dL], potassium 2.6 mEq/dL, free plasma metanephrines (MN) < 25 pg/mL [ref range: < or = 57 pg/mL], free normetanephrines (NMN) < 25 pg/mL [ref range: < or = 148 pg/mL, total free MN and NMN < 25 [ref range: < or = 205 pg/mL]. 24-hour urine free cortisol (UFC) was markedly elevated at 1854.1 mcg/24h [ref range: 3.1-50.0 mcg/24h], repeat 2186.2 mcg/24h, ACTH: 1.5 pg/mL [ref range: 7.2-63.6 pg/mL], confirming a diagnosis of ACTH-independent CS. Her case was complicated due to the adrenal nodule location. Patients with left adrenal nodules are at risk of developing inferior vena cava (IVC) syndrome during left adrenalectomies, especially in the third trimester, due to compression exerted by the uterus while lying on the right side during the procedure. She underwent an uncomplicated laparoscopic left adrenalectomy. Was started on hydrocortisone (HC) following surgery. There was concern for transient neonatal adrenal insufficiency at delivery. Measuring adrenal insufficiency in utero is challenging; however, estriol could serve as a surrogate marker of fetal adrenal function. Her estriol was normal, possibly indicating intact fetal adrenal activity. She had an uncomplicated vaginal delivery with no signs of adrenal insufficiency in her baby. Interestingly, she had a severely low alkaline phosphatase (ALP) which completely normalized soon after adrenalectomy. Clinical Lesson: This case depicts the challenges in managing CS in pregnancy and its repercussions regarding maternal, fetal, and delivery outcomes. Additionally, it highlights the importance of implementing a multidisciplinary approach, including obstetricians, endocrinologists, endocrine surgeons, and pediatricians, to ensure the optimization of therapeutic strategies. Presentation: Friday, June 16, 2023

THU660 Pericardial Effusion Secondary To Uncontrolled Hypothyroidism

Abstract Disclosure: G.D. Cain: None. D. Goldsmith: None. E. Ruel: None. Introduction: Hypothyroidism is an endocrine disorder that can effect multiple organ systems. Pericardial effusion is a rare but significant complication of hypothyroidism. Early diagnosis and management of the pericardial effusion is important to prevent progression to cardiac tamponade and hemodynamic instability. We present a case of pericardial effusion in a 62-year-old female with hypothyroidism and medication non-compliance. Case Presentation: A 62-year-old female with past medical history of hypothyroidism and hypertension presented to the emergency department due to altered mental status. Patient's family reported that she had a progressive decline in her speech for more than a month, until she became completely aphasic on day of presentation, which prompted them to call EMS. Patient had diminished exercise capacity, dyspnea on exertion, bilateral pedal edema over the past couple of months. Family also reported that the patient had been depressed and negligent of her health following the passing of her husband three years ago. She was not taking her medications including levothyroxine. In the ED, patient was hypertensive, blood pressure was 190/100. CT head stroke protocol did not show stroke. Chest x-ray showed massive cardiac enlargement. 2D-Echo confirmed a large pericardial effusion without cardiac tamponade. Thyroid function panel showed high TSH of 77, free T4 was undetectable. Pericardial effusion was most likely secondary to hypothyroidism. Myxedema coma was suspected due to patient's progressive mental deterioration. Patient also had myxedema of bilateral lower extremities. IV levothyroxine and hydrocortisone were started with improvement in her symptoms. A pericardial window was performed by the thoracic surgeon for her large pericardial effusion. Patient had large amounts of drainage from JP pericardial drain for more than two weeks following the surgery. JP drain was removed after the amount of drainage decreased to less than 100 ml a day. Cultures and cytology for malignancy were sent and all came back negative. Patient’s symptoms improved with treatment and the she was discharged on levothyroxine 137 mcg daily. Patient was counseled to take her medication regularly. Follow-up appointments were arranged with primary care provider and endocrinology. Discussion: Pericardial effusion is a rare complication of hypothyroidism, more likely to be seen in severe, untreated hypothyroidism such as in this case. The mechanism of pericardial effusion in hypothyroidism is through increased capillary permeability in heart and decreased lymphatic drainage of albumin, resulting in accumulation of fluid in the pericardial space. The effusion develops over time and patients can have large amounts of volume at the time of diagnosis without tamponade physiology. Early diagnosis is important to avoid tamponade and hemodynamic instability. Presentation: Thursday, June 15, 2023

THU013 A Rare Case of Ectopic Cushing’s Syndrome Caused By Metastatic Parotid Gland Carcinoma

Abstract Disclosure: E. Punni: None. I. Goyal: None. Introduction: Ectopic Cushing’s Syndrome (ECS) is a rare condition that accounts for only a small percentage of all Cushing’s syndrome cases. Although rare, the typical tumors associated with ECS are small cell lung carcinomas, bronchial carcinoids, pancreatic neuroendocrine tumors, medullary thyroid carcinomas, thymic carcinoids and pheochromocytomas. Metastatic acinic cell carcinoma (ACC) of the parotid gland has very rarely been associated with ectopic Cushing’s syndrome. Case Presentation: A 67-year-old Caucasian male with a known diagnosis of high grade metastatic ACC of the parotid gland presented to the hospital with progressively worsening proximal muscle weakness and bilateral lower extremity edema of 6 weeks duration. A month prior to presentation, he developed persistent hypokalemia requiring potassium supplementation and was diagnosed with new onset type 2 diabetes mellitus. Laboratory results on admission were remarkable for low potassium at 2.5 mEq/L (N: 3.5-5.3 meq/L), a random cortisol level of 107 mcg/dL (N: 6 - 30 mcg/dL), and an ACTH level of 485 pg/mL (N: 7.2 - 63.3 pg/mL). Subsequently, a 24-hour urine cortisol was > 1600 ug/24 hours (N 5-64 ug/24 hours). A 1 mg dexamethasone suppression test failed to suppress an 8 a.m. cortisol with level of 110.8 mcg/dl (N < 1.8 mcg/dl). Following, an 8 mg dexamethasone suppression test also failed to suppress 8 a.m. cortisol with a value of 100.8 mcg/dl. A MRI of the pituitary with and without contrast showed mild diffuse enlargement of the pituitary gland concerning for pituitary hyperplasia. At the time, ECS was highly suspected given the rapid course of patient’s presentation, known metastatic malignancy, and failed 8 mg dexamethasone suppression test. An inferior petrosal sinus sampling (IPSS) could not be performed given the patient’s guarded condition. Instead, a PET/CT Ga-68 DOTA-TATE scan obtained showed widespread DOTA-TATE avid malignancy concerning for neuroendocrine type tumor. Patient was a poor surgical candidate, therefore bilateral adrenalectomy could not be performed for the rapid treatment of ECS. Patient was started on a steroidogenesis inhibitor ketoconazole. However, he elected to undergo hospice care and passed away shortly after. Conclusion: This case is particularly interesting given that metastatic ACC of the parotid gland causing ECS is rare with only a few cases reported in the literature. This case highlights the importance of maintaining a high degree of suspicion for diagnosing ECS especially when patients present with rapid onset of signs and symptoms of Cushing’s syndrome and a known underlying malignancy. Recognizing less common causes of ECS may prompt early diagnosis and intervention. Presentation: Thursday, June 15, 2023

FRI503 HCG-induced And Autoimmune Hyperthyroidism In A Male Patient With HCG Secreting Germ Cell Tumor

Abstract Disclosure: A.H. Mohamed: None. M. Basina: None. Background: While Graves’ disease is the most common cause of hyperthyroidism, other rare causes include HCG secreting germ cell tumors. We describe a hyperthyroidism case with metastatic HCG secreting germ tumor and Graves’ disease. Case: 34 years old smoker male presented in 2016 with weakness and hypokalemia 2.7 mmol/L with concerns of paroxysmal periodic paralysis and was found to have hyperthyroidism consistent with Graves’ disease per work up (suppressed TSH <0.01- uIU/ml (ref 0.27-4.2 uIU/ml), elevated FT4 4.08 ng/dl (ref 0.93-1.7 ng/dl), elevated Free T3 15.0 (ref 2.3 - 4.0 pg/ml), and (thyroid stimulating immunoglobulin (TSI) 354 (ref 0 - 139 %). He was started on methimazole 20 mg that he did not use and lost follow up. In May 2022, he presented with expressive aphasia and seizures along with 4 months of right scrotal swelling, generalized lymphadenopathy, and hemoptysis episodes. He had intermittent palpitations, 30 Ibs weight loss and eye bulging. On exam he was tachycardic 120 bpm, enlarged thyroid, brisk reflexes and mild tremors. His eye exam was significant for stare, lid lag, proptosis and bilateral lid edema. His labs were remarkable for suppressed TSH <0.01 uIU/ml, FT4 >7.7 ng/dl , Total T3 394 ng/dl (80 - 200 ng/dl)Thyrotropin receptor antibodies (TRAb) 29 IU/L (ref 0-1.75 IU/L), TSI 4.8 (ref <1.3 index), Thyroglobulin Ab 5.8 IU/ml (ref <4IU/ml), TPO-Ab >900 (ref <9.0) and HCG >1,000,000 (ref <2mIU/ml). Thyroid US showed diffuse heterogeneous enlargement of the thyroid with increased vascularity and without a discrete well-defined nodule. His lymphadenopathy work revealed high grade stage III C non-seminomatous mixed germ tumors (60% teratoma and 40% choriocarcinoma) with metastasis to brain, lungs, liver, spine, iliac and sacral bones. He was started on methimazole (MMI) 20 mg TID, propranolol 40 mg TID, dexamethasone 2 mg q6hrs and chemotherapy. His thyroid labs improved in 3 days to FT4 2.83 ng/dl and TT3 93 ng/dl while TSH and HCG were unchanged. He was discharged on MMI 25 mg daily. Over 3 months, he subsequently underwent right orchiectomy, cyber knife radiation of the brain metastatic lesions and 4 chemotherapy cycles. His HCG levels fluctuated (1,000,000->3980-> 120,181) as well thyroid function test (TSH <0.01-> 5.1->0.01 uIU/ml, FT4 1.02->0.65->5.7 ng/dl and TSI 6.4 ). He required multiple methimazole adjustments. Discussion: Co-occurrence of HCG secreting germ tumors and Graves’ hyperthyroidism is rare. HCG can bind TSH receptors and in high levels has thyrotropic effects. The interplay between HCG thyrotropic effects and thyroid autoimmunity can be challenging and use of both binding and functional bioassays maybe helpful. Close thyroid function monitoring and frequent adjustment of thioamides are needed as patients undergo treatment of the primary HCG source as well as long term follow up. Presentation: Friday, June 16, 2023

SAT174 Doege-Potter Syndrome In A Patient With Solitary Fibrous Tumor Of The Lung

Abstract Disclosure: N. Mon: None. S.S. Krishnasamy: None. Doege-Potter Syndrome is a non-islet cell tumor hypoglycemia (NICTH) due to a solitary fibrous tumor (SFT) with overexpression of Insulin-like growth factor 2 (IGF2). We report a case of refractory hypoglycemia in a patient with SFT of the lung. Case presentation: A 67-year-old homeless male with hypertension, chronic smoker presented with altered mental status after his blood glucose was found 34mg/dl by EMS. His confusion improved after D50W. He could not recall the event but he reported that he missed his dinner prior to the event. He denied taking any antidiabetic medications. He was diagnosed with left lung solitary fibrous tumor without metastases a week ago after he presented to ER with dyspnea, cough and wheezing. CT chest reported a 21x15.6x18.7 cm lesion with mass effect on the left hemidiaphragm, mediastinum shift to the right and occluded left lower lobe bronchi. Core needle biopsy of the mass revealed SFT with necrosis. He was discharged with Medrol dosepak 4mg daily for presumptive treatment of COPD exacerbation and a plan for elective surgery. While in the ER his BG improved to 136mg/dl after D50W. Few hours later BG dropped to 42mg/dl and D10W infusion was started. Physical exam was unremarkable except bilateral pedal edema. Work up showed BG 42mg/dl, Insulin 3.3 (2.6-24.9 uIU/mL), C peptide 0.39 (1.1-4.4ng/mL), Proinsulin 4.7 (<18.8pmol/L), beta-hydroxybutyrate <0.05 (0.02-0.27mmol/l), 8AM cortisol 10.7 (7.0-25.0 ug/dl), albumin 3.2mg/dl, HbA1c 5.8%, IGF1 53ng/mL (34-245ng/mL), IGF2 203ng/mL (267-616ng/mL) with a IGF2:IGF1 ratio of 3.8. In our patient, the IGF2:IGF1 ratio was not >10 since his IGF 2 was suppressed by the recent steroid use. The rest of the clinical and biochemical features strongly suggested NICTH. His preoperative evaluation showed new onset HFrEF with volume overload. He underwent staged percutaneous coronary interventions with stents placement. He was given hydrocortisone 50mg q6H then to q8H during hospitalization and discharged home with hydrocortisone 40mg TID. He underwent radical resection of lung tumor after optimization of his cardiac condition. He was on stress dose of steroid during surgery. Since he was on high dose steroid for > 3months, hydrocortisone dose was tapered slowly after the surgery. Final biopsy reported solitary fibrous tumor extending to parenchymal staple line. Tumor cells are positive for CD34 and STAT6, and negative for desmin, SMA, and SOX10. He recovered uneventfully and was discharged home with prednisone 5mg daily and follow up in endocrine clinic. Conclusion: NICTH is a rare paraneoplastic syndrome characterized by low levels of insulin, C-peptide, proinsulin, and beta-hydroxybutyrate. A ratio of IGF-2/IGF-1 more than 10 confirms NICTH; however, normal ratio cannot exclude the diagnosis and factors such as low IGFBP 3 or steroid therapy for other conditions need to be considered. Surgery is the mainstay of treatment. Presentation: Saturday, June 17, 2023

THU449 An Unexpected Case Of Cutaneous Leukocytoclastic Vasculitis Following Zoledronic Acid Infusion

Abstract Disclosure: B. Zahedi: None. Z.S. Wallace: None. M.M. Cote: None. E.W. Yu: Grant Recipient; Self; Amgen Inc. Background: Zoledronic acid is a potent amino-bisphosphonate frequently used in the treatment of osteoporosis. We report a case of small-vessel vasculitis with cutaneous manifestations occurring after zoledronic acid infusion, previously not reported in the literature. Clinical case: A 58-year old woman with a history of sleeve gastrectomy 1.5 years prior was treated for bone protection with an intravenous infusion of zoledronic acid (Zol) 5mg. This was the patient’s first exposure to a bisphosphonate medication. Within minutes of the infusion, she developed a single episode of diffuse watery diarrhea. Although GI symptoms resolved quickly, within a few hours she experienced generalized myalgias and skin tenderness in her abdomen and extremities, for which she took NSAIDs. These symptoms progressed in severity over the next 3-5 days, manifesting as bilateral hand and lower extremity edema and erythema; no fever/chills/sweats, mucocutaneous ulcers, or urinary symptoms were present. By Day 5, she developed palpable purpura without blanching above the ankles and extending to the knees. By Day 7, prednisone 60mg was started. Prior to Zol, labs showed sufficient 25-hydroxyvitamin D, normal calcium, renal and liver function. On Days 10 and 14, labs revealed AST and ALT, twice and thrice the upper limit of normal respectively. Other notable labs included normal UA, Ca, WBC, eGFR, nonelevated ESR/CRP, and a negative work up for an underlying infectious, inflammatory, ANCA-associated, hypocomplementemic or autoimmune process. Cutaneous findings resolved within 3 weeks of Zol, and transaminitis and edema resolved by week 4. In the setting of a negative lab workup and typical palpable purpura, the patient was diagnosed with leukocytoclastic cutaneous vasculitis (LCV), likely induced by Zol (Naranjo score of 5 consistent with probable adverse drug reaction). Although IgA-mediated Henoch-Schonlein Purpura (HSP) cannot be definitively ruled out in absence of a skin biopsy, the patient did not exhibit other clinical features of HSP, and the timeline is highly suggestive of an idiosyncratic drug reaction whereby Zol acted as a hapton to stimulate an immunologic vasculitis response. Up to 50% of patients with LCV have systemic involvement (renal, GI, pulmonary, cardiac or CNS). Cutaneous LCV usually carries a favorable prognosis after treatment with glucocorticoids. On review of the literature, no prior cases of bisphosphonate-induced LCV have been reported, although there are few cases of giant cell arteritis, uveitis and urticarial vasculitis with bisphosphonate therapy. Conclusions: This is the first case demonstrating cutaneous manifestations consistent with leukocytoclastic vasculitis due to zoledronic acid infusion, which was successfully managed with NSAIDs and glucocorticoids. Clinicians should be aware that LCV may be a rare hypersensitivity complication of Zol. Presentation: Thursday, June 15, 2023