Initial Manifestation Of Sarcoidosis Research Articles

Real-Life Utilization of Criteria Guidelines for Diagnosis of Cardiac Sarcoidosis (CS).

Despite the increasing recognition of cardiac involvement in systemic sarcoidosis, the diagnosis of cardiac sarcoidosis (CS) remains challenging. Our aim is to present a comprehensive, retrospective case series of CS patients, focusing on the current diagnostic guidelines and management of this life-threatening condition. In our case series, patient data were collected retrospectively, including hospital admission records and rheumatology and cardiology clinic visit notes, detailing demographic, clinical, laboratory, pathology, and imaging studies, as well as cardiac devices and prescribed medications. Cases were divided into definite and probable CS based on the 2014 Heart Rhythm Society guidelines as well as presumed CS based on imaging criteria and clinical findings. Overall, 19 CS patients were included, 17 of whom were diagnosed with probable or presumed CS based on cardiac magnetic resonance imaging (CMR) and/or cardiac positron emission tomography using 18F-Fluorodeoxyglucose (PET-FDG) without supporting endomyocardial biopsy (EMB). The majority of CS patients were male (53%), with a mean age of 52.9 ± 11.8, with CS being the initial manifestation of sarcoidosis in 63% of cases. Most patients presented with high-grade AVB (63%), followed by heart failure (42%) and ventricular tachyarrhythmia (VT) (26%). This case series highlights the significance of utilizing updated diagnostic criteria relying on CMR and PET-FDG given that cardiac involvement can be the initial manifestation of systemic sarcoidosis, requiring prompt diagnosis and treatment to prevent morbidity and mortality.

SARCOIDOSIS AND HYPERCALCIURIA: A STONE COLD INDICATION FOR TREATMENT

TOPIC: Diffuse Lung Disease TYPE: Fellow Case Reports INTRODUCTION: Sarcoidosis may cause renal disease by several different mechanisms. Symptomatic nephrolithiasis is a rare occurrence as the initial manifestation of sarcoidosis. Hypercalciuria may occur commonly in sarcoidosis and warrants treatment. There is lack of data supporting screening for hypercalciuria. Herein we present a patient with asymptomatic sarcoidosis until she developed nephrolithiasis with a normal serum calcium but an elevated urine calcium. CASE PRESENTATION: 62 year old woman with a history of diabetes mellitus II and obesity was followed in the pulmonology clinic for abnormal chest imaging. She had previously presented to the emergency department for chest pain that lead to a chest radiograph and Chest CT scan. CT scan showed mediastinal adenopathy and pulmonary nodules. Bronchoscopy was performed with biopsies which revealed non necrotizing granulomas consistent with sarcoidosis. Patient had a normal spirogram, and no other extrapulmonary manifestations that would warrant treatment for sarcoidosis. Serum calcium and albumin were normal. 25 hydroxy vitamin D levels were 23 ng/ml. She did take over the counter Vitamin D supplements. Later she developed nephrolithiasis. Analysis of the stone revealed 65% calcium oxalate monohydrate and 15% calcium oxalate dihydrate. A 24 hour urine calcium showed 328 mg of calcium per volume of urine. Patient had a history of diabetes mellitus so treatment with steroids was avoided and she was started on 10 mg of methotrexate. Follow up urine calcium levels are pending. DISCUSSION: While there are guideline recommendations to screen for hypercalcemia with a serum calcium level and for measuring vitamin D levels for replacement, there is scant literature on screening for hypercalciuria in the setting of normal calcium levels, despite hyercalciuria being the most common renal manifestation of renal sarcoidosis. The incidence of renal disease in sarcoidosis varies from 1.1% to 9.7% with hypercalciuria occurring in up to 50% while hypercalcemia may occur in only 10-20% of cases, making hypercalciuria the most common renal manifestation of sarcoidosis. Hypercalciuria is the result of increasing calcium load filtered at glomerulus as a result of decreased PTH secretion secondary to elevated circulating calcitriol levels leading to a diminished tubular reabsorption of calcium. Hypercalciuria predisposes to calcium oxalate nephrolithiasis. Approximately 10 % of patients with sarcoidosis have 1 symptomatic stone. For 1 % of these patients it may be the initial presentation. Approximately 2.7% of sarcoidosis patients have asymptomatic stones. CONCLUSIONS: Hyercalciuria is the most common renal manifestation of sarcoidosis, yet it is not commonly screened for in asymptomatic sarcoidosis patients. Timely treatment may attenuate renal impairment secondary to sarcoidosis. It is suggested that hypercalciuria in the setting of sarcoidosis always be treated. REFERENCE #1: Adam R. Berliner, Mark Haas, Michael J. Choi, Sarcoidosis: The Nephrologist's Perspective, American Journal of Kidney Diseases, Volume 48, Issue 5,2006, Pages 856-870, ISSN 0272-6386, https://doi.org/10.1053/j.ajkd.2006.07.022. REFERENCE #2: Thomas KW, Hunninghake GW. Sarcoidosis. JAMA. 2003 Jun 25;289(24):3300-3. doi: 10.1001/jama.289.24.3300. PMID: 12824213 DISCLOSURES: No relevant relationships by Jonathan gwizdala, source=Web Response Grant for research relationship with Sanofi Please note: January 2021 Added 04/20/2021 by Daniel Ouellette, source=Web Response, value=Grant/Research No relevant relationships by Rajaninder Sharma, source=Web Response

Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis.

Sarcoidosis is a multi-system disease of unknown etiology characterized by the formation of granulomas in various organs. It affects people of all ethnic backgrounds and occurs at any time of life but is more frequent in African Americans and Scandinavians and in adults between 30 and 50 years of age. Sarcoidosis can affect any organ with a frequency varying according to ethnicity, sex and age. Intrathoracic involvement occurs in 90% of patients with symmetrical bilateral hilar adenopathy and/or diffuse lung micronodules, mainly along the lymphatic structures which are the most affected system. Among extrapulmonary manifestations, skin lesions, uveitis, liver or splenic involvement, peripheral and abdominal lymphadenopathy and peripheral arthritis are the most frequent with a prevalence of 25–50%. Finally, cardiac and neurological manifestations which can be the initial manifestation of sarcoidosis, as can be bilateral parotitis, nasosinusal or laryngeal signs, hypercalcemia and renal dysfunction, affect less than 10% of patients. The diagnosis is not standardized but is based on three major criteria: a compatible clinical and/or radiological presentation, the histological evidence of non-necrotizing granulomatous inflammation in one or more tissues and the exclusion of alternative causes of granulomatous disease. Certain clinical features are considered to be highly specific of the disease (e.g., Löfgren’s syndrome, lupus pernio, Heerfordt’s syndrome) and do not require histological confirmation. New diagnostic guidelines were recently published. Specific clinical criteria have been developed for the diagnosis of cardiac, neurological and ocular sarcoidosis. This article focuses on the clinical presentation and the common differentials that need to be considered when appropriate.

Gastric Sarcoidosis: An Atypical Presentation of a Rare Disease

Sarcoidosis is a multisystemic granulomatous disease characterised by the histologic evidence of granulomas in various organs. Clinically discernible gastric sarcoidosis is extremely rare, reported in less than 1% of the patients. We present a case of gastric sarcoidosis with atypicial manifestations and without current pulmonary disease. The patient was admitted for gastric fullness, anorexia, and weight loss. She had a history of migraine and nodular goiter. Family history revealed bladder carcinoma in the mother and gastric carcinoma in the uncle. Initial laboratory findings and chest x-ray were normal. Biopsies from the gastric mucosa revealed noncaseating granulomas with focal multinucleate giant cells. Six months after the patient was admitted for dry cough and fatigue. Chest x-ray and CT revealed diffuse nodules in the upper and middle lung zones. Transbronchial biopsy demonstrated noncaseiting granulomas compatible with sarcoidosis. The patient was started on mehtylprednisolone and had a significant improvement in her symptoms. This case is an unique paradigm for the exceptional presentation of sarcoidosis. Isolated extrapulmonary organ involvement without simultaneous lung disease may be the initial manifestation of sarcoidosis. Meticulous follow-up for sarcoidosis is required for patients presenting with atypical symptoms and noncaseiting granulomas in any extrapulmonary organ.

Hypercalcemia and AKI as the Initial Manifestation of Sarcoidosis

Hypercalcemia and AKI as the Initial Manifestation of Sarcoidosis

Central canal enhancement and the trident sign in spinal cord sarcoidosis.

We report an enhancement pattern in 9 patients with spinal cord sarcoidosis (SCS) with subacute onset (<4 weeks) myelitis as the initial manifestation of sarcoidosis. Presenting symptoms included numbness in 9, urinary complaints in 7, and weakness in 6. Examination revealed abnormal proprioception in 8, spastic quadriparesis/paraparesis in 6, and sensory level in 6. MRI showed central canal enhancement alone (11%) or in combination with dorsal-subpial enhancement1 (89%) (figure 1), often resembling a trident head on axial sequences (figure 2). Lung biopsies in 7 patients confirmed sarcoidosis; both patients without biopsies had hilar adenopathy. Central canal enhancement and the trident sign in subacute myelitis should raise suspicion for SCS.

Massive Pleural Effusion as an Initial Manifestation of Sarcoidosis

Massive Pleural Effusion as an Initial Manifestation of Sarcoidosis

Granulomatous Interstitial Nephritis Presenting as Hypercalcemia and Nephrolithiasis.

We report a case of acute kidney injury as the initial manifestation of sarcoidosis. A 55-year-old male was sent from his primary care physician's office with incidental lab findings significant for hypercalcemia and acute kidney injury with past medical history significant for nephrolithiasis. Initial treatment with intravenous hydration did not improve his condition. The renal biopsy subsequently revealed granulomatous interstitial nephritis (GIN). Treatment with the appropriate dose of glucocorticoids improved both the hypercalcemia and renal function. Our case demonstrates that renal limited GIN due to sarcoidosis, although a rare entity, can cause severe acute kidney injury and progressive renal failure unless promptly diagnosed and treated.

Acute arthritis as initial presentation of sarcoidosis: Significance of chest X-ray

Sarcoidosis is a multisystem granulomatous disease that predominantly affects the lungs. It usually presents with fatigue and respiratory findings. The rate of arthritis in sarcoidosis is 15-25%. Arthritis as initial manifestation of sarcoidosis has been reported rarely in the literature. In present paper we highlighted the importance of sarcoidosis and conventional chest X-ray in differential diagnosis of acute arthritis.

Cervical Myelopathy from Spinal Sarcoidosis as the Unexpected Initial Presentation for Systemic Sarcoidosis

This is a case which highlights unique diagnostic challenges in the evaluation of a previously healthy patient presenting with a myelopathy initially most concerning for malignancy. However, timely recognition and nonoperative therapy of unexpected spinal sarcoidosis with corticosteroidal therapy was crucial in averting the sequelae of undiagnosed or misdiagnosed neurosarcoidosis, which can be devastating and life-threatening. Diagnosis is challenging due to significant similarities in clinical and roentgenographic findings of spinal sarcoidosis with infection, inflammation, and malignancy. Our case demonstrates that diagnosis of spinal sarcoidosis is especially elusive when it is the initial manifestation of sarcoidosis.

SARCOIDOSIS PRESENTING AS BRAINSTEM ISCHEMIC STROKE

Sarcoidosis is characterized by noncaseating granulomas that involve the nervous system in 5% of patients.1,2 Stroke is uncommon despite small vessel inflammation, but when present, is usually supratentorial and rarely the first symptom of neurosarcoidosis.3 We present two patients with brainstem strokes as the initial manifestation of sarcoidosis. ### Case reports. #### Case 1. A 35-year-old African American man suddenly developed left-sided weakness in September 2001. He had mild hypertension diagnosed 6 months earlier, but no other risk factors. MRI 6 days later demonstrated increased T2 signal and early enhancement in the pons; no diffusion imaging was performed. Stroke evaluation was not performed and he was treated with aspirin and clopidogrel. In July 2002, he developed lethargy and urinary incontinence. MRI without contrast showed communicating hydrocephalus and a shunt was placed; CSF was not examined. Over the next year, the patient required six shunt revisions for presumed idiopathic hydrocephalus. Over the subsequent 2 years, he developed bilateral deafness, ataxia, and memory impairment. Our initial examination in March 2004 revealed a blood pressure of 140/90 mm Hg, short term memory loss, hearing impairment, and left greater than right spastic hemiparesis. Brain and spine MRI showed encephalomalacia of the mid-pons and leptomeningeal enhancement (figure, A). CSF showed 24 leukocytes/mm …

Changes on optic nerve with sarcoidosis patients

Abstract Purpose Sarcoidosis is a rare autoimmune disorder. Since in 20% of the cases optical manifestations represent the first sign of a systemic disease and that 75% of sarcoidosis patients are under the age of 40, we considered it appropriate to perceive the manifestations of sarcoidosis on the optic nerve and to notice the consequences for the vision. Methods The examination was being carried out on 32 patients who had come for an ophthalmological check‐up in the Eye Clinic of the Clinical Centre of Montenegro for the first time, and who had been made sarcoidosis diagnosis. With the patients showing the obvious signs of neurosarcoidosis with the spreading over the optic nerve, additional diagnostic examinations were being carried out: FA, ICG, NMR, EEG, VEP, lumbar punction, as well as endocrinological examinations. The patients were being monitored during the following year, with monthly control check‐ups. Results Optical manifestations within the framework of the basic disease were found with 17 patients, whilst optical symptomatology was the first sign of the disease with 4 patients. The changes on the optic nerve were noticed in various forms and they appeared either as the initial manifestation of sarcoidosis or at any time during the duration of the disease. Conclusion The changes found on the optic nerve within the framework of sarcoidosis were numerous and variable. PNO edema, papillitis, granuloma PNO, retrobulbar neuritis, are only part of clinical manifestations which appear upon the optic nerve being affected by sarcoidosis.

A case of sarcoidosis presenting as a non‐specific intramedullary lesion

Neurosarcoidosis occurs in 5-15% of sarcoidosis cases. Approximately 50% of patients with neurosarcoidosis present with a neurological disease at the time sarcoidosis is first diagnosed. Spinal sarcoidosis is rare. We report the case of a 61-year-old man with a highly aspecific intramedullary lesion as the first manifestation of sarcoidosis. One year after the onset of neurological symptoms, the high levels of angiotensin-converting enzyme and the results of a total body gallium scan and bronchoalveolar lavage supported the diagnosis of sarcoidosis. Isolated single reports indicate that spinal neurosarcoidosis may be the initial manifestation of sarcoidosis. In our case, magnetic resonance imaging of the dorsal spine showed a largely aspecific lesion. Neurosarcoidosis should be considered in the differential diagnosis of intramedullary cord lesion with leptomeningeal enhancement; a systematic search for evidence of sarcoidosis should be mandatory in all cases for a correct diagnosis and early treatment.

Concurrent Intermediate Uveitis and an Enhancing Intracranial Lesion as the Initial Manifestation of Sarcoidosis

Posterior segment involvement has been described to be associated with central nervous system involvement in sarcoidosis as a result of direct sarcoid tissue infiltration or mass effect of a cerebral lesion. However, isolated intermediate uveitis occurring concurrently with central nervous system involvement prior to extensive systemic disease is rare. We describe a patient with neuro-ophthalmic manifestations of intermediate uveitis and an enhancing basal ganglia lesion at initial presentation, in the absence of extensive systemic disease. He was treated with high-dose systemic steroids which was progressively tailed down over 6 months. There was prompt resolution of vitritis with good preservation of visual acuity. The difficulties of the initial diagnosis of sarcoidosis and the indications for initiation of steroid therapy are illustrated. We use this case to emphasise the need for a high clinical suspicion of sarcoidosis in the presence of similar unusual and seemingly unrelated combinations of neurological manifestations so as to facilitate the prompt institution of appropriate treatment when indicated.

Aspiration biopsy of nodular sarcoidosis of the muscle No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.

The nodular form of muscular sarcoidosis is rare and the condition is often confused with a soft tissue neoplasm. We present a case of nodular muscular sarcoidosis of the right calf in a 52-yr-old woman which was diagnosed by thin-needle aspiration biopsy (TNAB). In this patient, a calf mass was the initial manifestation of sarcoidosis. Contrast-enhanced computed tomographic scans of the calf showed a mass with peripheral ring-like enhancement. T2-weighted magnetic resonance images demonstrated a mass with central hypointensity and marked peripheral hyperintensity. TNAB revealed noncaseating epithelioid cell granulomas in association with multinucleated giant cells. Combined with the radiologic findings, TNAB is considered a useful modality for the diagnosis of nodular muscular sarcoidosis. Diagn. Cytopathol. 2002;26:109–112; DOI 10.1002/dc.10055 © 2002 Wiley-Liss, Inc.

Aspiration biopsy of nodular sarcoidosis of the muscle

The nodular form of muscular sarcoidosis is rare and the condition is often confused with a soft tissue neoplasm. We present a case of nodular muscular sarcoidosis of the right calf in a 52-yr-old woman which was diagnosed by thin-needle aspiration biopsy (TNAB). In this patient, a calf mass was the initial manifestation of sarcoidosis. Contrast-enhanced computed tomographic scans of the calf showed a mass with peripheral ring-like enhancement. T2-weighted magnetic resonance images demonstrated a mass with central hypointensity and marked peripheral hyperintensity. TNAB revealed noncaseating epithelioid cell granulomas in association with multinucleated giant cells. Combined with the radiologic findings, TNAB is considered a useful modality for the diagnosis of nodular muscular sarcoidosis.

Sarcoidosis of the Pancreas: Case Report and Review of the Literature

Sarcoidosis involving the pancreas is rare. Patients can present with symptoms that mimic pancreatic cancer. We report a case of a male patient with clinical and radiographic findings suggestive of pancreatic cancer as the initial manifestation of sarcoidosis.

Conjunctival deposits as an initial manifestation of sarcoidosis

PURPOSE: To report conjunctival deposits as an initial manifestation of sarcoidosis. METHODS: Case reports. RESULTS: Two patients with bilateral bulbar conjunctival white deposits underwent conjunctival biopsy. The biopsy specimens showed noncaseating granulomas with prominent Schaumann bodies. Serum angiotensin-converting enzyme levels were increased in both patients. The first patient had abnormal results of pulmonary function tests. Sarcoidosis was diagnosed in both patients. CONCLUSIONS: Conjunctival deposits may represent the initial clinical manifestation of sarcoidosis.

Lacrimal bypass surgery in patients with sarcoidosis

Purpose: To examine the results of lacrimal bypass surgery in patients with sarcoidosis. Methods: Patients with sarcoidosis who underwent dacryocystorhinostomy (DCR) or conjunctivodacryocystorhinostomy (CDCR) in two practice settings from 1986 through 1995 were identified and their medical records reviewed. Results: Twelve patients, of whom eight were women, underwent bilateral DCR or CDCR to treat nasolacrimal duct obstruction associated with sarcoidosis. The initial diagnosis of sarcoidosis was established in four patients from a biopsy specimen obtained during DCR. The ages of the patients at diagnosis of sarcoidosis ranged from 39 to 64 years (mean, 49.6 years; median, 45.5 years), whereas their ages at the time of surgery ranged from 42 to 72 years (mean and median, 55 years). The average duration of postoperative follow-up evaluation was 44 months (median, 38.5 months; range, 10 to 82 months). All patients received local corticosteroids postoperatively, and nine patients (75%) were treated with prednisone. Of the 24 lacrimal procedures, 23 (95.8%) were patent to irrigation at the last follow-up examination, and all patients were asymptomatic. Conclusion: Lacrimal drainage obstruction may be the initial manifestation of sarcoidosis, and tissue obtained during DCR may help to establish the diagnosis. A successful surgical outcome may require intensive and occasionally long-term therapy with local and systemic corticosteroids.

A case of sarcoidosis revealed in the course of IgA nephropathy

A 36 year old man, who had been proteinuric for 14 years due to immunoglobulin A (IgA) nephropathy, was admitted because of an acute exacerbation in renal dysfunction with hypercalcemia. He had presented with aortic regurgitation and increased pulmonary marking by chest X-ray, but laboratory examinations had failed to make an exact diagnosis. On admission, noncaseating epithelioid granulomas were disclosed by muscle and skin biopsies. Ophthalmological evaluation revealed old uveitis and retinal changes consistent with sarcoidosis. In this case, IgA nephropathy was thought to be the initial manifestation of sarcoidosis that developed latently. Sarcoidosis should be considered in a differential diagnosis of IgA nephropathy.