Bilateral Leg Swelling Research Articles

Carbon dioxide (CO2) as an alternative contrast agent in percutaneous transluminal angioplasty procedures for chronic limb-threatening ischemia patients with chronic kidney disease

Background: Chronic limb-threatening ischemia (CLTI) is a very morbid illness that significantly reduces quality of life. The severity of this disease may necessitate revascularization or amputation. Arteriography is frequently performed for the planning of revascularization. Patients with compromised kidney function need alternative contrast agents. CO2 may be utilized in lower extremity vascular diagnostics and intervention, especially in patients at risk for contrast-induced nephropathy (CIN). Case Illustration: An 80-year-old female with bilateral leg pain and leg swelling had an ulcer on her toe for 2 weeks. She had a history of intermittent claudication for 1 year, hypertension for 5 years, kidney stones for 2 years, and chronic kidney disease for 1 year. She underwent an ECG, laboratory, USG, DUS, and CT-angiography examination. From her laboratory result, her initial creatinine was 5.02 mg/dL (eGFR 8 mL/m/1.73 m2). After good hydration, it became 1.28 mg/dL (eGFR 39 mL/m/1.73 m2). After a CT-angiography procedure, her creatinine level was increased (3.7 mg/dL; eGFR 10.9 mL/m/1.73 m2). She was diagnosed with CLTI Rutherford V Fontain 3 left lower extremity. She suggested doing an angioplasty procedure with a safer contrast agent. An angioplasty procedure was done at RSSA Malang with CO2 contrast. After the procedure, her leg pain improved, and her creatinine didn’t elevate. Conclusion: CO2 angiography might be used as a safe alternative contrast medium in patients with CLTI, which benefits the preservation of renal function and prevents limb amputations. Keyword: chronic limb-threatening, chronic kidney disease, CO2 angiography, contrast-induced nephropathy, peripheral artery disease

Disseminated AIDS-associated Kaposi sarcoma without cutaneous involvement: a case report and review of literature.

Disseminated AIDS-associated Kaposi sarcoma (KS) without cutaneous lesions is rare and can present in varying ways. Diagnosis is even more challenging now when incidence of KS is on the decline. A high index of suspicion is required for early diagnosis and treatment. Therefore, the medical literature should be made aware of any manifestations of KS that can occur without the typical cutaneous lesions. A 23-year-old presented with worsening cervical lymphadenopathy, recurrent cough and bilateral leg swelling of a month duration. Examination revealed features of pericardial effusion, pulmonary fibrosis, necrotizing cervical lymphadenopathy and the presence of pityriasis rotunda at the periumbilical region. Patient was diagnosed human immunodeficiency virus (HIV) positive 6months before she presented and was placed on antiretroviral therapy. Histology confirmed AIDS-associated KS. However, patient died before commencement of chemotherapy. The clinical course of disseminated AIDS-associated KS without cutaneous lesions can be atypical and aggressive. It is important to include KS inthe differential diagnosis of cases with atypical or persistence/recurrence of clinical symptoms in spite of treatment especially in HIV patients.

Prevalence of Pulmonary Hypertension in Liver Cirrhosis Patients: A Cross-Sectional Analytical Study.

Liver cirrhosis (LC) is a common complication of chronic liver disease. Its prevalence has increased markedly over the last few years. With liver cirrhosis comes cardiovascular morbidity and mortality. It is important that the detection of the abnormalities by echocardiography be given priority, as this can change the clinical outcome of these patients with cardiovascular abnormalities in liver cirrhosis. This study aims to determine the prevalence of pulmonary hypertension in LC patients. A cross-sectional analytical study was carried out at JUTH (Jos University Teaching Hospital) over a period of one year. We recruited 210 adult patients with liver cirrhosis from the gastroenterology clinic and wards for this study. Data from these patients were collected with questionnaires administered by the interviewer and analysed using SPSS 23 statistical software (IBM Corp., Armonk, NY). The data obtained are presented in tables and charts. Categorical variables were expressed as proportions and frequencies, while continuous data were expressed as the median, mean, and standard deviation. Pulmonary hypertension was found in 30.5% of the participants, with mild pulmonary hypertension being the most common. No one had severe pulmonary hypertension. There was an increased risk of developing pulmonary hypertension in patients with coughs, easy fatigability, bilateral leg swelling, abdominal swelling, and ascites (P<0.05). The result showed that there is a high prevalence of pulmonary hypertension in patients with liver cirrhosis.

OA06 Behçet's associated myositis: an unusual cause of exercise-induced leg pain

Abstract Introduction This case describes a 29-year-old gentleman of Cypriot heritage who presented with exercise-induced bilateral lower leg pain and swelling which made it difficult for him to cycle. Symptoms progressed over months to calf pain at rest accompanied by fatigue. He subsequently presented with central visual loss in the right eye, followed by the left. The second episode of visual loss coincided with development of oro-genital ulceration and, following rheumatology review, a diagnosis of Behçet’s disease was made. The case is significant in terms of the chronicity and nature of presenting symptoms which is not typically described in this disease. Case description A 29-year-old gentleman presented with exercise-induced bilateral lower leg pain and swelling for which he was reviewed in emergency care on four occasions. He received treatment for presumed cellulitis and underwent US-doppler studies, excluding thromboembolic disease. MRI of the lower legs was requested given lack of diagnosis with ongoing symptoms. This demonstrated bilateral muscle oedema in the antero-lateral compartments and lateral gastrocnemius heads, prompting rheumatology referral. Prior to attending rheumatology, the patient developed bilateral sequential visual loss. This was attributed to right acute macular neuroretinopathy, followed by significant left posterior pole choroidal ischaemia 2 months later. Each presentation was associated with transient low-grade anterior uveitis, vitritis, and the presence of a pre-papillary vitreous infiltrate. Initial treatment included a six-week oral prednisolone taper from 60mg daily. He reported a progressive leg pain which initially occurred during cycling but later affecting his walking. He was of Cypriot heritage, with no family history of autoimmune disease. A focused history revealed oral ulceration and an acne-like pustular rash over the back. His gait was antalgic with minimal toe-off phase. Investigations demonstrated raised ESR and CRP, negative autoimmune immunology, negative myositis ENA, normal CK, and positive HLA-B51. Given these features, a provisional diagnosis of Behçet’s was made. Nerve conduction studies were requested to consider whether changes could be secondary to denervation. They displayed non-specific S1 neurogenic changes and small tibial motor amplitudes. Subsequent MRI lumbar spine excluded radiculopathy. Repeat CK remained within normal limits. Muscle biopsy was sought and performed with a clinical suspicion of atypical myositis which has previously been reported in Behçet’s. At the second episode of visual loss, he was admitted under ophthalmology and treated with intravenous methylprednisolone and azathioprine. The case was discussed with our regional centre of excellence for Behçet’s and infliximab at 5mg/kg was commenced. Discussion This case was notable for the complexity and atypical presenting features with calf pain induced by exercise likely due to atypical myositis (biopsy report still awaited at time of submission). More classical Behçet’s disease symptoms including oro-genital ulceration were significantly later. The case highlights the importance of multidisciplinary working and involving specialist centres especially when rapid escalation to biologic treatment is required. Leg symptoms are persistent but improved despite ongoing treatment with prednisolone and azathioprine with the recent addition of infliximab. Visual symptoms have stabilised and skin involvement has settled. Management Rationale At the time of first review in rheumatology clinic, high-dose steroid treatment had already been initiated; therefore, expediting the investigative aspect of his management felt most appropriate, particularly given that myositis seemed to be the predominant feature at that time. In joint consultation with neurophysiology and neurology, putting the EMG and MRI findings in context, MRI of the lumbar spine was felt necessary to exclude discogenic disease causing denervation oedema. Unfortunately, visual symptoms progressed rapidly after this, requiring admission, which precluded an earlier opportunity for biopsy but leading to quick authorisation and organisation of treatment with infliximab. Key learning points This case highlights an atypical presentation of Behçet’s disease including associated likely atypical myositis. Causes of exercise-induced leg pain in a young, fit patient need to be considered: The need for multidisciplinary discussion both across specialties and with regional centres is key in order to progress with diagnostics and treatment In sharing this case for discussion, we wish to highlight possible causes of exercise-induced calf pain and a pathway for investigation of this. Our patient had atypical myositis as the cause for his symptoms in the context of multi-system disease. Both his myositis and ophthalmology presentations were somewhat atypical but multidisciplinary working, along with the development of some further symptoms, were helpful in making the diagnosis.

E018 COVID-19 vaccine-induced myositis

Abstract Background/Aims Through the COVID pandemic there have emerged reports of autoimmunity or new rheumatic diseases presenting in patients after they had COVID-19. This is thought to be caused by cross-reactivity of the COVID-19 spike protein to human antigens. Given the use of mRNA COVID-19 vaccinations which express the spike protein we might expect to see presentation of new rheumatic diseases following their use. We discuss a case where this appears to have occurred. Methods Our patient is a 24-year-old male with mixed phenotype acute leukaemia who had been treated with allogenic stem cell transplant and was currently in remission. He presented with fevers, palpitations, myalgia and bilateral arm and leg swelling. Symptoms began the day after receiving the first dose of an mRNA COVID-19 vaccination (Pfizer/BioNTech.) There were no other symptoms or recent change in medications. Physical examination revealed tender oedema in his forearms, biceps and thighs bilaterally with sparring of the hands. He had reduced power with shoulder (MRC 3/5), elbow (4), wrist (4+) and hip (4) movements. Observations revealed tachycardia and fevers up to 40C. Results Laboratory studies showed markedly elevated C-reactive protein (202), creatinine kinase (6697) and troponin (593) whilst investigations for infection were negative. An autoimmune panel was positive for anti-PM-SCL-75-Ab. An electrocardiogram showed sinus tachycardia. Echocardiogram was normal. Bilateral upper limb dopplers revealed no deep vein thrombus. An MRI of his thighs showed diffuse symmetrical oedema within the muscles, in keeping with an inflammatory myositis. A quadricep muscle biopsy showed evidence of MHC class 1 up-regulation, suggesting an inflammatory process. In addition, there were numerous macrophages evident in the endomysium. While this can be seen in graft-versus-host disease (GVHD), they would usually be found in the perimysium. After discussion between haematology, rheumatology and neurology, this was felt to be a case of vaccine induced myositis and myocarditis. Autoimmune myositis was thought to be less likely due to the relative sparing of the hands and the absence of Raynaud’s phenomenon. 1 gram of intravenous methylprednisolone was then given for 3 days. The patient had a marked response with defervescence, improving laboratory markers, improved myalgia and decreased limb swelling. The patient was stepped down to a reducing regime of prednisolone and discharged. Due to relapse whilst weaning he has started on mycophenalate mofetil and rituximab and now continues to improve. Conclusion There are case reports of myositis following COVID-19 vaccination but our patient’s case is complicated by the differential diagnosis of GVHD and concurrent myocarditis. Ongoing work is needed to clarify the exact link between vaccination and the presentation of a new inflammatory myositis, but it is important to recognise and start treatment early in order to preserve muscle bulk and ensure recovery. Disclosure L. Thompson: None. T. Nadin: None. J. Meiring: None. S. Thomas: None. R. Kumari: None. S. Carter: None. M. Akil: None. A. Alfred: None. N. Morley: None.

A Patient with Third-Space Fluid Loss.

A 64-year-old woman with underlying hypertension treated with oral perindopril presented with a history of worsening abdominal distension, bilateral leg swelling, and exertional dyspnea over 4 months. In addition, she also complained of intermittent palpitations, weight loss, and anorexia. Her blood pressure was 125/68 mmHg, and she was tachycardic (114 beats per minute) with a low-grade fever of 37.5°C (99.5°F) and good oxygen saturation on room air. Physical examination suggested the presence of third-space fluid loss, evidenced by ascites, right-sided pleural effusion, and bilateral lower limb edema. There was no cardiac murmur or evidence of chest infection on examination. Initial laboratory investigations revealed pancytopenia, a decreased serum folate concentration, and severe hypoalbuminemia with an albumin to globulin (A:G) ratio of 0.13 (reference interval 1–2). The rest of the initial investigations showed normocalcemia, no renal impairment, and no electrolyte imbalance (Table 1). A chest X-ray showed a right-sided pleural effusion, which confirmed the need for a work-up for third-space fluid loss. An echocardiogram revealed an ejection fraction of 59% (reference interval 54–74%), and grade 1 left ventricular hypertrophy with diastolic dysfunction.

Commentary on A Patient with Third-Space Fluid Loss.

This is an interesting clinical case highlighting the value of body fluid testing to assist in diagnosing a complex patient presenting with multiple effusions. The clinical presentation of unilateral pleural effusion is usually more suggestive of exudative causes, although bilateral effusion with right-side predominance is observed in transudative effusions. Bilateral leg swelling is observed more often with transudates, such as those caused by heart or liver failure (1). Severe hypoalbuminemia is frequently caused by renal failure associated nephrotic syndrome and/or end stage liver failure associated with reduced synthetic output. Renal and liver impairment were inconsistent with clinical findings until the globin predominance prompted protein electrophoresis, which returned a diagnosis of myeloma as an explanation for severe hypoalbuminemia. It is possible that severe hypoalbuminemia may have contributed to the transudative pleural effusion as well as ascites. The successful chemotherapeutic treatment for myeloma resolved the ascites and presumably the pleural effusion as serum albumin normalized. Lack of measurement of serum or plasma NT-ProBNP makes heart failure a questionable contributor to the transudative effusion, and follow-up may be warranted posttreatment to definitively rule out cardiac disease.

167: AN ADOLESCENT MALE WITH CONGESTIVE HEART FAILURE FROM A RARE CAUSE

Introduction: A sinus of Valsalva aneurysm is an abnormal dilatation of the aortic root located between the aortic valve annulus and the sinotubular junction. It can either be congenital or acquired. The right coronary sinus is most commonly involved, followed by the noncoronary sinus. Rupture of the sinus of Valsalva aneurysm is extremely rare in children. Description: A 16-year-old previously healthy male was admitted with 1 month of worsening fatigue, bilateral leg swelling and orthopnea. He was hypertensive and tachycardic with a grade 3 holosystolic murmur over the precordium. The rest of his physical examination was normal. A chest x-ray showed cardiomegaly and an EKG showed ventricular bigeminy and inverted T waves. His pro-BNP was elevated to 1880 pg/mL but troponin and inflammatory markers were normal. A transthoracic echocardiogram (TTE) showed a ruptured aortic noncoronary sinus of Valsalva aneurysm into the right atrium with left to right shunting, aortic root dilatation and aortic valve regurgitation. A cardiac CT confirmed the TTE findings. He underwent surgical repair. Intraoperatively, a large fenestrated aneurysmal tissue was noted and was excised, with the defect repaired using two layers of autologous pericardial patches. A transesophageal echocardiogram (TEE) showed no residual defect and no aortic insufficiency. He was extubated the next day, but postoperative course was complicated by an accelerated junctional rhythm with ventricular bigeminy that was successfully treated with an esmolol infusion and later transitioned to atenolol. Discussion: Sinus of Valsalva aneurysm is present only in 0.09% of the general population. The mean age of diagnosis is between 30-45 years, and it is usually asymptomatic unless it ruptures. The right ventricle followed by the right atrium are the most common locations of the rupture. Cardiac catheterization and angiography is the gold standard for diagnosis but a TTE is the first line. Cardiac CT and MRI are supplemental tests. Ruptured aneurysms or unruptured symptomatic aneurysms indicate surgical repair. With the onset of heart failure symptoms, arrhythmias and aortic insufficiency diagnosed on a TTE, a surgical repair with patch closure was opted for our patient with a good postoperative outcome.

COVID-19 Associated Atypical Hemolytic Uremic Syndrome

Introduction: Atypical hemolytic uremic syndrome (atypical HUS) is a complement-mediated thrombotic microangiopathy (TMA) that affects multiple organs including the kidneys. We present a case of 37-year-old male with recent COVID-19 infection who presented with epistaxis. His blood work was consistent with TMA and acute kidney injury (AKI). Hemodialysis and plasmapheresis (PLEX) were initiated for presumed thrombotic thrombocytopenic purpura (TTP) but after excluding ADAMTS-13 deficiency, eculizumab was started for possible atypical HUS. Renal biopsy and genetic testing ultimately confirmed diagnosis of atypical HUS. Case presentation: A 37-year-old male with past medical history of recent COVID infection presented to the emergency room with complaint of epistaxis. Physical exam showed mild epistaxis from right naris and bilateral leg swelling. Initial laboratory findings were hemoglobin (Hb) 5 g/dL, platelets 70 B/L, Lactate dehydrogenase (LDH) 581 IU/L, haptoglobin < 10 mg/dL, creatinine (Cr) 36.80 mg/dL, blood urea nitrogen (BUN) 232 mg/dL, potassium (K) 6.6 mmol/L, calcium (Ca) 6 mg/dL and normal prothrombin time (PT) and partial thromboplastin time (PTT). Complement C3 levels were low but normal C4 levels. Peripheral blood smear showed schistocytes confirming TMA. He was started on both PLEX and hemodialysis (HD) along with prednisone 80 mg daily. PLEX was stopped on day 4 after normal ADAMTS-13 levels resulted, helping rule out TTP. Based on history and laboratory findings, diagnosis of atypical HUS was favored and eculizumab was started. He received meningococcal vaccine and prophylactic penicillin while on eculizumab. Hemodialysis was continued with eventual improvement of his labs to Hb 7, platelets 119, LDH 339, haptoglobin < 10, Cr 9.07, BUN 85, K 5.8, and Ca 8.0. He was discharged on bimonthly dose of eculizumab and hemodialysis three times a week. Two months later, his labs were Hb 10.4, platelets 184, LDH 490, haptoglobin < 10, Cr 11.60, BUN 82, K 5.2, and Ca 9.2. PT and PTT remained normal. Given persistence of laboratory findings, renal biopsy and genetic testing for complement proteins was done. Kidney biopsy showed diffuse global renal microangiopathy with negative C3. Genetic testing showed a heterozygous C3 variant, homozygous deletion of CFHR1-CFHR4, negative factor H autoantibodies (FHAA), and single nucleotide variant in C5 gene. Discussion: Atypical HUS is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and AKI. The complement system is a part of the immune system that enhances the ability of phagocytic cells to clear microbes and damaged cells. Atypical HUS is caused by dysregulation of the alternative complement pathway that leads to microvascular thrombosis. Various mutations in complement and its regulatory proteins have been discovered in atypical HUS patients. Heterozygous C3 variants result in persistently low C3 levels and cause severe disease. Most individuals with a homozygous deletion of CFHR1 and/or CFHR3, CFHR4 genes have a pathogenic role in the development of the anti-factor H autoantibodies. These autoantibodies interfere with the binding of CFH to the C3 convertase and are associated with defective CFH-dependent cell protection. Some case reports of COVID-19 patients showed that complement activation was significantly stronger in lung alveolar septa, hyaline membranes, and in alveolar lumina via the lectin and alternative complement pathways. Treatment of atypical HUS is symptomatic but in severe disease eculizumab or ravulizumab have roles by binding to the complement protein C5, inhibiting its cleavage to C5a and C5b, and preventing formation of terminal complement complex C5b-9. Patients on these therapies are at risk of meningococcal disease, thus it is recommended to get meningococcal vaccines and prophylactic penicillin for the duration of therapy. Our patient had homozygous deletion of CFHR1-CFHR4 genes with no factor H autoantibodies. However, he did have C3 variant with gain of function mutation which caused overt activation of alternative pathway as indicated by his low C3 levels with normal C4 levels. He responded partially to eculizumab and discussion on possible kidney transplant has been started.

Abstract 11714: High-Output Heart Failure in Multiple Myeloma: Novel Approach to a Challenging Diagnosis

High-output heart failure (HOHF) is a rare complication of multiple myeloma and is believed to be related to arteriovenous shunting in osseous myeloma lesions. Timely diagnosis is crucial, as these patients respond best to aggressive treatment of the underlying malignancy. In this case, a 51-year-old man with multiple myeloma complicated by extensive osseous disease was admitted from oncology clinic for several weeks of worsening dyspnea, early satiety, and bilateral leg swelling. His exam was notable for signs of volume overload, and the BNP was elevated to 1444 pg/ml. An echocardiogram showed new right ventricular dysfunction. After the patient failed to respond to escalating doses of intravenous diuretics, right heart catheterization was performed and revealed elevated biventricular filling pressures with evidence of high cardiac output (Fick CO 17.1 LPM, Fick CI 7.9 LPM/m 2 ). In a novel diagnostic approach, 5.1 mCi of technetium-99m-labeled macroaggregated albumin was injected into the aorta at the time of left heart catheterization and then whole body planar images were obtained in anterior and posterior projections. The scan revealed evidence of lower extremity arteriovenous shunting with a proportion of 6% (Fig. 1). He was started on lenalidomide and pulse-dose dexamethasone with improvement in diuretic response and resolution of heart failure symptoms. A follow-up echocardiogram showed normalization of right ventricular size and function. He eventually underwent successful allogeneic stem cell transplant during the admission. Although Tc-99m MAA is routinely used in the diagnosis of hepatopulmonary syndrome, this is, to our knowledge, the first report of its use to investigate myeloma-related HOHF in the clinical setting. This technique was previously utilized successfully to experimentally correlate between shunting values and cardiac indices. It may be of use in a wider population of patients with HOHF in whom occult shunting is suspected.

Abstract 14984: Transient Constrictive Pericarditis: Pericardiectomy to the Rescue

Case Presentation: A 47 years old male presented with progressive dyspnea, bilateral leg and abdominal swelling, weight gain, and intermittent low-grade fever. He had a history of hypertension and chronic lymphocytic pericarditis complicated by an episode of constrictive pericarditis. Exam showed jugular venous pressure of 10 cmH2O, pericardial knock, and abdominal distention. Inflammatory markers were elevated (CRP 87.3 mg/dL, ESR 29 mm/hr). Echo showed abnormal increase in septal longitudinal motion and Doppler interrogation of the hepatic veins showed diastolic flow reversal at the end of expiration. Cardiac MRI showed pericardial thickening and circumferential delayed pericardial enhancement. Patient was diagnosed with transient constrictive pericarditis (TCP) and was treated with ibuprofen, colchicine, and prednisone and had improvement in his symptoms and inflammatory markers. However, he experienced another recurrence 6 months later and underwent pericardiectomy. Pathology showed organized fibrosis overlying the parietal fibrosa layer, mild neovascularization and perivascular chronic inflammation, confirming the diagnosis of TCP. After pericardiectomy, patient had significant symptomatic improvement and returned to his baseline activity level. Postoperative echo showed normalization of annulus longitudinal motion and cardiac MRI showed near resolution of pericardial delayed enhancement. He was successfully tapered off of his anti-inflammatory regimen. Discussion: TCP is a sub-type of constrictive pericarditis characterized by pathological findings of pericardial edema, acute or subacute inflammation, and fibrin deposition, as opposed to the fixed pericardial fibrosis and calcification seen in constrictive pericarditis. Constriction may resolve spontaneously, and anti-inflammatory therapy is the mainstay of treatment but in refractory cases like these, pericardiectomy comes to the rescue.

PMON117 IGF-1 Testing: Diagnostic Pitfalls

Abstract Introduction Acromegaly is a rare endocrine disorder, with an incidence of 5.3/million/year, that results from persistent hypersecretion of growth hormone (GH), which stimulates hepatic secretion of insulin-like growth factor-1 (IGF-1). The best single test for the diagnosis of acromegaly is measurement of serum IGF-1; however, the result must be interpreted with caution, with understanding of potential pitfalls. Here we present a case of falsely elevated IGF-1 levels. Case Description A 43-year-old female with medical history of prediabetes, sleep apnea, and headaches was referred to endocrine clinic for evaluation of possible acromegaly. Patient had been evaluated by her primary care physician for frequent headaches, unintentional weight gain, and bilateral leg and hand swelling. Patient endorsed being unable to wear her wedding ring for a few years. Initial laboratory evaluation by primary care physician 3 months prior revealed normal thyroid function, prolactin, FSH, LH, random cortisol, but elevated IGF-1 of 310 ng/mL (normal 71-258 ng/mL), with negative pregnancy test. Brain MRI at that time revealed a pineal gland cyst but no pituitary abnormality. During her initial endocrine visit, IGF-1 was repeated, which was again elevated but improved to 283 ng/mL (normal 71-258 ng/mL). Other pertinent labs included a normal random ACTH of 18 pg/mL (normal &amp;lt;47 pg/mL), and pm cortisol of 2.4 mcg/dL (normal 2.9-17.3 mcg/dL). Physical examination was notable for a few light purple striae on abdomen, acanthosis nigricans, and mild limb edema, but no macrognathia, enlarged hands or feet. Given patient's lack of typical features of acromegaly, normal pituitary imaging, and spontaneous IGF-1 improvement, it was decided to postpone GH measurements with oral glucose tolerance test but repeat IGF-1 in 6 months. Patient was able to follow up 11 months after initial visit at which patient denied any worsening symptoms. At that time, repeat laboratory study showed normal IGF-1 of 231. Given spontaneous normalization of IGF-1 levels, no further laboratory testing or imaging studies were recommended, and patient was instructed to follow up in 11-12 months. Discussion Prompt diagnosis of acromegaly is crucial. However, it is important to note IGF-1 could be falsely elevated. A retrospective review by Erickson et. al of 2747 unique patients with IGF-1 elevation, 117 (4%) were found to have false-positive results following a review of the clinical data. In addition to acromegaly, other causes of elevated IGF-1 levels include high protein and high glycemic-index diet, pregnancy, hyperthyroidism, IGF-1 assay problems, and some rare tumors (carcinoids) secreting IGF-1. Discovery of "falsely" elevated IGF-1 may lead to unnecessary testing and physician visits. Therefore, clinicians should consider retesting patients after some time interval if spurious IGF-1 is suspected, before aggressively pursuing more extensive investigations. Presentation: No date and time listed

P13 A case of Kikuchi-Fujimoto disease triggered by an episode of bacterial cellulitis, associated with features of SLE

P13 A case of Kikuchi-Fujimoto disease triggered by an episode of bacterial cellulitis, associated with features of SLE

P38 The gut that leaks

Introduction/BackgroundSystemic lupus erythematosus is a chronic inflammatory autoimmune disease which can affect multiple organ systems. Symptoms of intestinal involvement in SLE may vary from common and non-specific such as heartburn, oral ulcers and dysphagia to less common but more serious including abdominal pain due to serositis and intestinal vasculitis.Description/MethodA 20 year old girl was referred to the gastroenterology team for the management of ascites. Her past medical history included SLE diagnosed aged 10, class IV lupus nephritis, autoimmune haemolytic anaemia secondary to SLE, Haemoglobin H, gastrostomy feeding due to poor growth.At the time of the transition from paediatric to adult service her SLE was under good control. She was on Prednisolone 6 mg on alternate days, Mycophenolate (MMF) 750 mg BD, Septrin 240 mg OD and Enalapril 2.5 mg OD. After the transition, MMF was reduced to 500 mg OD. The rest were unchanged. Serum total protein was 57 (60-80), serum albumin 32 (35-50). Weight was 41.2 kg.Ten months after the transition, she presented with bilateral leg swelling and subsequently tense ascites over 3 weeks. Serum total protein reduced to 31, albumin 13.24 hour urine total protein was 0.15 (0-0.1), urine volume 1.7 L. Serum creatinine 20 µmol/L and eGFR >90. Urine PCR was 16 mg/mmol.Total protein in ascites drain fluid was <20 g/L, albumin <10 g/L, glucose 5.7 mol/L. There were no malignant cells and no growth on enrichment culture.She was referred to the dietitians who advised that her oral intake was adequate.There was no evidence of heart failure on echocardiogram.Small bowel meal showed oedematous bowel wall but no abnormalities in terminal ileum. Coeliac serology was negative.CT abdomen and pelvis showed normal appearances to the liver, hepatic and portal veins; no features of Budd-Chiari syndrome; normal size kidneys; incidental finding of non-occlusive right internal jugular thrombus and moderate lymphadenopathy in mesenteric and retroperitoneal regions.She developed swelling in the right calf. Doppler ultrasound confirmed major DVT with occlusive thrombus extending to distal superficial femoral veins. Clots in inferior vena cava and portal veins were excluded.Discussion/ResultsDuodenal biopsy confirmed villous oedema and dilated lymphatics consistent with changes seen in protein losing enteropathy. She was referred to Rheumatology. Prednisolone was increased to 15 mg. The response was rapid with reduction in ascites. MMF was increased to maximum tolerable dose 750 mg AM and 500 mg PM.Hb had been persistently low around 50 during this illness. She had longstanding hypocomplementaemia. C3 dropped to 0.37 (baseline 0.6). C4 remained low around 0.04. IgG was 5.9 (baseline >7). Serology showed DsDNA was 5 (normal range 0-15), positive ANA, trace positive RNP and negative Rheumatoid factor and APL antibodies. CRP was 10.Abdominal lymphadenopathy was felt to be due reactive changes due to active lupus.With increased steroids and immunosuppressants, she improved significantly both clinically and biochemically within a few weeks. Hb rose to over 90, serum albumin > 25 and serum protein above 50 by the end of 3 months after treatment escalation.Warfarin was stopped upon completion of 6 month anticoagulation.On repeat CT, previous abdominal lymphadenopathy had resolved completely. Splenomegaly measuring 17.6 cm was noted. This was discussed in haematology MDT and thought to be due to the haemoglobinopathy and haemolysis.Haemolysis continued to be a challenge. She remained on relatively high dose steroids varying between 20 mg and 10 mg over the years. Vitamin D was prescribed. Weight was very low but stable just above 38 kg.Protein Losing Enteropathy (PLE) is rare but recognised in SLE. It typically occurs in young women with clinically severe disease with multi organ involvement. Clinical presentation is characterised by the onset of profound oedema, hypoalbuminaemia in the absence of nephrotic range proteinuria. Severe diarrhoea is present in 50% of the patients. It is important to investigate and exclude other causes of hypoalbuminaemia (e.g cardiac failure, liver diseases, malnutrition) and PLE.Key learning points/ConclusionPLE can be the initial manifestation of SLE.Lupus nephritis and PLE can be present successively or simultaneously in a lupus patient.Stringent diagnostic evaluation is required to exclude other mimicking conditions.Multidisciplinary team involvement is crucial in the management of patients with multi-organ involvement.PLE associated with SLE usually responds well to corticosteroids. However additional immunosuppressant(s) are generally required to treat other SLE symptoms as well as to help with the steroid reduction regime.As well as treating the underlying disease, advice from dietitians is important to improve and maximise the nutritional status of these patients.A clinically significant hyper-coagulable state is reportedly attributed to PLE associated with SLE. Therefore, VTE prophylaxis especially in hospitalised patients is recommended. Moreover, clinicians should have high index of suspicion for VTE in these patients with multiple risk factors to arrange early appropriate investigations and treatment plan.

Screening for Severe Illness at Diagnosis Has the Potential to Prevent Early TB Deaths: Programmatic Experience From Karnataka, India.

Due to the coronavirus disease (COVID-19) pandemic and its associated response, TB deaths increased for the first time in a decade. In any potentially fatal illness, an assessment of severity is essential. This is not systematically done for adults with TB, mostly due to a lack of policy and/or limited availability of diagnostic and clinical capacity. We developed a screening tool using simple and easily measurable indicators that can be used by paramedical TB program staff to quickly identify people with severe illness. During October-November 2020 in Karnataka, India, the paramedical program staff from 16 districts screened people with TB (aged ≥15 years) notified by public facilities for "high risk of severe illness," which was defined as the presence of any of the following indicators: (1) body mass index (BMI) ≤14.0 kg/m2; (2) BMI ≤16.0 kg/m2 with bilateral leg swelling; (3) respiratory rate >24/minute; (4) oxygen saturation <94%; (5) inability to stand without support. In this cohort study, we determined the incidence of program-recorded early deaths (within 2 months) and its association with high risk of severe illness. Of 3,010 people with TB, 1,529 (50.8%) were screened at diagnosis/notification, of whom 537 (35.1%) had a high risk of severe illness. There were 195 (6.5%, 95% CI=5.7, 7.4) early deaths: 59 (30.2%) within a week and 100 (51.3%) within 2 weeks of treatment initiation. The incidence of early deaths was significantly higher among those with high risk of severe illness (8.9%) at diagnosis compared to those without (3.8%) [adjusted relative risk: 2.36 (95% confidence interval=1.57, 3.55)]. To conclude, early deaths were especially high during the first 2 weeks and strongly associated with a high risk of severe illness at diagnosis/notification. Screening for severe illness should be explored as a potential strategy to end TB deaths.

Screening adults with tuberculosis for severe illness at notification: programme experience from Gujarat, India.

National tuberculosis (TB) programmes in high-burden countries do not systematically assess severity at diagnosis followed by referral for inpatient care. Due to limited capacity, comprehensive assessment of all TB patients is not practical. In three districts of Gujarat (West India) in June 2021, we determined the feasibility of screening for severe illness by paramedical staff of public facilities and the burden of 'high risk for severe illness' at notification among adults (≥15y of age) with TB. In this cross-sectional study, the screening criteria for high risk for severe illness was the presence of any one of the following: body mass index (BMI) ≤14.0kg/m2, BMI ≤16.0kg/m2 with bilateral leg swelling, respiratory rate >24/min, oxygen saturation <94% or inability to stand without support. We summarized the feasibility indicators and burden using proportion (95% confidence interval [CI]) and median, as applicable. Of 626 notified adults, 87% were screened. The median time interval for screening was 3d from notification and all indicators were collected in 97% of patients. The burden of high risk for severe illness was high, at 41.6% (95% CI 37.5 to 45.8). High burden and feasibility (high coverage, acceptable time interval and minimal missing data) makes a strong case for routine screening of severe illness.

Hypokalaemia in Type-4 Renal Tubular Acidosis: A Rare Presentation

Proximal/Type-2 Renal Tubular Acidosis (RTA) occurs due to impairment of bicarbonate absorption in the proximal tubule and is mainly characterised by hypokalaemia and metabolic acidosis. There are various causes of proximal RTA like genetic, cystinosis, Wilson’s disease, drugs and toxins, vitamin D deficiency, hyperparathyroidism, amyloidosis. Vitamin D deficiency is one of the uncommon causes and is more common in elderly. Hereby, authors present a case report of a diabetic and hypothyroid 30-year-old female, who presented with chronic limb weakness, myalgia and bilateral leg swelling. After detailed history taking, clinical examination and laboratory investigations, she was diagnosed with nephrotic syndrome and proximal RTA associated with vitamin D deficiency. She had hypokalaemia with acidosis initially and was managed with diuretics, Angiotensin Converting Enzyme (ACE) inhibitors, vitamin D and potassium supplementation, after which, she developed hyperkalaemia with acidosis. She was evaluated for hyperkalaemic RTA and its common causes and was diagnosed with hyperkalaemic RTA associated with diabetes. Thus, treatment of type-2 RTA due to vitamin D deficiency, led to unmasking of underlying type-4 RTA due to diabetic nephropathy.

Abstract 12913: Under Pressure: An Unusual Case of Pulmonary Hypertension and Pericardial Effusion

A 50-year-old male smoker with hypertension presented with subacute bilateral leg swelling, weight gain, and dyspnea. Exam revealed tachycardia, jugular venous distention, peripheral edema, diminished breath sounds, and pulsus paradoxus. Labs were notable for elevated creatinine and leukocytosis. ECG showed sinus tachycardia and low voltages. CXR showed left hemithorax opacification. TTE revealed normal LV function, septal flattening, severely dilated RV with reduced function, RVSP 81 mmHg, and a large pericardial effusion with exaggerated respirophasic variation in mitral inflow but no chamber collapse. The elevated RVSP with RV failure raised concern for acute pulmonary embolism (PE). CT chest excluded PE but revealed a large mediastinal mass compressing both pulmonary arteries (PA) and large left pleural effusion. Pulmonary angiogram demonstrated a totally occluded left PA and severe stenosis of the right PA. Right heart catheterization showed RAP 24, RV 83/21, PA 84/29 (mean 53), intrapericardial pressure 15 mmHg, and low cardiac index (1.6 L/min/m2). Pericardiocentesis was performed with close monitoring by PA catheter. Removal of 500cc fluid was well tolerated with stable hemodynamics, cardiac output, and intracardiac pressures. Pericardial fluid cytology and lymph node biopsy demonstrated small cell lung cancer. Cautious diuresis was initiated. After one cycle of chemotherapy, repeat CT chest showed reduced extrinsic compression of the right PA with improved lung perfusion. Repeat TTE showed dramatic improvement with normal RV size and function, RVSP 22 mmHg, and trivial pericardial effusion. Pericardial effusion in the setting of severe PH is associated with poor outcomes and presents both diagnostic and therapeutic challenges. Classic findings of tamponade (e.g. right sided chamber collapse) may be masked by elevated right heart pressures. Compression of the PA by a mediastinal mass is an unusual cause of PH and right heart failure that can mimic acute PE. This rare clinical scenario presents the following challenges: (1) diuresis may precipitate tamponade, (2) pericardiocentesis may enable an already enlarged RV to acutely dilate and thus worsen RV failure, and (3) there is uncertainty regarding the role for PA stenting.

Eosinophilic fasciitis: experience with a patient and review of the potential mimics.

Bilateral lower limb swelling is a common clinical scenario with a wide differential diagnosis. We present a young man with gradually worsening bilateral leg swelling, who was diagnosed with eosinophilic fasciitis. A 20 year old Hispanic male presented with a six week history of bilateral lower limb pain and swelling, later involving the upper limbs, but sparing the hands, feet and face. He had initial pitting, followed by non-pitting oedema and had a positive 'groove sign'. With peripheral eosinophilia, a clinical diagnosis of eosinophilic fasciitis was suspected and was later confirmed on histology. He improved with prednisolone initially and is currently maintained on tapering prednisolone alongside methotrexate.

Irritable Infant with Bilateral Leg Swelling.

Irritable Infant with Bilateral Leg Swelling.