Rare Presentation Of Sarcoidosis Research Articles

A Rare Presentation of Sarcoidosis: Dysphagia, Pancytopenia, and Acute Renal Failure

Sarcoidosis is a multisystem inflammatory chronic disorder that can virtually affect any organ system in the body. Most commonly affected organs are the intrathoracic structures with 90% of the reported cases involving the lungs. Esophageal involvement in sarcoidosis is extremely rare. Involvement of the esophagus and kidney along with hematological involvement is extremely uncommon in the same patient. Here, we present a case of a 58-year-old gentleman with a similar rare presentation. The patient presented with shortness of breath, productive cough, fatigue, and difficulty in swallowing, along with a weight loss of 20-30 pounds over three months. Laboratory workup was significant for leukopenia (2900 K/UL), serum creatinine level of 2.7 mg/dL (baseline: 1.2-1.7), and raised angiotensin-converting enzyme level at 187 nmol/ml/min. Chest X-ray showed bilateral widespread fine reticulonodular opacities, chest CT showed extensive bilateral reticulonodular opacities throughout the lung parenchyma, and fine-needle aspiration cytology of the right lung showed noncaseating granulomas. No fungal or acid-fast organisms were identified, and no evidence of malignancy was seen. Special stains for fungal (Grocott's methenamine silver and periodic acid-Schiff) and acid-fast organisms (acid-fast bacilli (AFB) and fluorescent AFB) were negative. Esophagogastroduodenoscopy (EGD) with gastric biopsy showed acute and chronic inflammation and no intestinal metaplasia, dysplasia, or malignancy was identified. Bronchoalveolar lavage was done, which showed macrophages (74%), neutrophils (6%), eosinophils (3%), and lymphocytes (17%), and was negative for malignant cells. QuantiFERON and AFB sputum/Mycobacterium tuberculosis polymerase chain reaction were negative. The patient was initially started on intravenous fluids and calcitonin, which significantly improved renal function and the calcium status of the body. Then prednisone 40 mg daily was started, which improved swallowing and breathing. After a week, prednisone was changed to 20 mg daily and was continued at the time of discharge.

P039 Muscle wasting & weakness: a rare presentation of sarcoidosis

Background/Aims Sarcoidosis is a rare multisystem disease characterised by the presence of noncaseating granulomas. It most commonly affects the lungs though can affect any other organ system. Rarely, it can manifest as an acute myopathy. We describe a case of a patient presenting with muscle weakness and constitutional symptoms who was eventually diagnosed with sarcoidosis. Methods A 48-year-old male with a background of lumbar spondylosis and BPH, presented with a 6-week history of progressive upper and lower limb weakness, myalgia and reduced mobility. He also described an 18-month history of progressive fatigue, drenching night sweats and 10-kilogram weight loss. His symptoms meant he was unable to work as a firefighter. Examination demonstrated profound muscle wasting and reduced power in the proximal muscles of his upper and lower limbs. There was no evidence of rash, synovitis or lymphadenopathy. Blood tests showed a normocytic anaemia (Hb 100 g/L) and raised C-reactive peptide (180 mg/L) and erythrocyte sedimentation rate (100 mm/hour). The creatine kinase ranged between 20-42 units/litre. He had a weakly positive anti-nuclear antibody (1:80). The remaining autoantibody screen was negative including ENA, DSDNA, ANCA, rheumatoid factor and anti-CCP. Complement proteins were unremarkable. Furthermore, an extended myositis panel revealed no myositis-specific or myositis-associated antibodies. Serum calcium and angiotensin-converting enzyme (ACE) levels were normal. Blood cultures and virology screen including for HIV, hepatitis B, hepatitis C, CMV, EBV, COVID-19 and respiratory viruses were all negative. A chest radiograph was also unremarkable. Results He subsequently underwent electromyography which revealed generalised myopathy. An MRI of the lower limb proximal musculature showed evidence of muscle oedema worse on the right-side but no definitive evidence of myositis. A PET-CT followed revealing FDG-avid generalised lymphadenopathy and polyarticular uptake, but little uptake in the skeletal muscles. He underwent an external iliac lymph node core biopsy which demonstrated multiple noncaseating granulomas and lymphadenitis. Cultures for Tuberculosis were negative and there was no evidence of a lymphoproliferative disorder. A muscle biopsy was desired but not possible due to lack of availability because of the COVID-19 pandemic. The patient was diagnosed with sarcoidosis and commenced on three pulses of intravenous methylprednisolone followed by a weaning regimen of high-dose oral prednisolone and subcutaneous methotrexate. This resulted in a sustained improvement in his symptoms and normalisation of inflammatory markers. Conclusion Symptomatic myopathy is present in only 0.5-2.5% of sarcoidosis patients. This unique case highlights the heterogeneity of this disease and the vital role different diagnostic modalities play in achieving the correct diagnosis. It is also pertinent that the lymphadenopathy, found incidentally via imaging, led to the diagnosis. Although notoriously a diagnosis of exclusion, this case emphasises the importance of considering sarcoidosis even in the absence of respiratory symptoms, a raised ACE or hypercalcaemia. Disclosure C. Tomlinson: None. A. Khan: None. D. Mukerjee: None. N. Bhadauria: None.

Intramedullary spinal cord involvement: a rare presentation of sarcoidosis.

Sarcoidosis is a systemic granulomatous disease of uncertain etiology, which predominantly affects the lungs, eyes, lymph nodes, and skin. Nervous system involvement (neurosarcoidosis) occurs in approximately 5–10% of patients, but spinal cord involvement is very rare, affecting <1% of patients. We present a rare case of intramedullary sarcoidosis.

Exclusive abdominal lymphadenopathy as a rare presentation of sarcoidosis

Sarcoidosis is a systemic granulomatous disease. The disease’s immunopathogenic mechanism is the activation of a cell mediated immune response. Lung is the most affected organ, but up to 30% of patients can have extrapulmonary manifestations. Case report: A 21-year-old male presented with a history of nausea, vomiting after meals, epigastric and periumbilical pain for approximately 6 months. Hyporexia and loss of 35 kilograms in 7 months were present, in addition to intermittent low nocturnal fever. His computed tomography (CT) scan (Figure 1) of the abdomen showed the presence of multiple, enlarged mesenteric lymph nodes with homogenous shape, measuring up to 2.0 cm in diameter, extending from the upper abdomen to the pelvis. Histopathologic findings of this lymph nodes showed a sarcoid appearance (Figure 2). Lymphoma, tuberculosis and other infections were excluded through immunohistochemical analysis. Treatment with prednisone was started initially at a dose of 40mg and after 5 weeks the patient were asymptomatic. One year and three months after the onset of treatment, corticosteroid dose reduction was attempted 3 times, with all of them leading to disease relapses. Treatment was optimized with 60mg of prednisone (for 2 weeks) plus 100mg of azathioprine daily. Discussion: The prevalence of gastrointestinal system involvement with clinical manifestations is 0,1 to 0,9%. However, the incidence of subclinical involvement can be higher. Enlarged lymph nodes is present in approximately 30% of cases. Treatment is indicated in sarcoidosis for patients with damage organ function or symptomatic disease and the first-line therapy is corticosteroid.

Scalp Sarcoidosis Presenting as Cicatricial Alopecia

Sarcoidosis is a granulomatous condition that has a highly variable presentation. One rare presentation of sarcoidosis is cutaneous scalp sarcoidosis. Usually scalp sarcoid presents as a scarring alopecia, but it can be nonscarring. The presence of sarcoidal lesions on the scalp is associated with systemic disease, as are other cutaneous manifestations of sarcoidosis. The authors present the case of a 64-year-old woman with a history of sarcoidosis who presented with alopecia and hypopigmented patches on her scalp. She also had papular sarcoid lesions on her upper back and a history of pulmonary involvement, which is consistent with previous reports in the literature. The condition subsequently improved with topical clobetasol propionate.

DON’T LET THE DRY MOUTH FOOL YOU

SESSION TITLE: Signs and Symptoms of Chest Disease SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Sarcoidosis is a well-known though poorly understood disease process with a multitude of manifestations. Clinicians should be aware of the diverse manifestations, as any evidence of active sarcoidosis requires consideration for treatment. We present a case of Mikulicz syndrome, a rare presentation of sarcoidosis in which the parotid and lacrimal glands are infiltrated by granulomas with resulting dry mouth, dry eyes and occasionally recurrent fevers. CASE PRESENTATION: The patient is a thirty-seven-year-old African-American female with a history of pulmonary and dermatologic sarcoidosis. She was diagnosed after a skin biopsy revealed noncaseating granulomas in the setting of chronic dyspnea with bilateral hilar lymphadenopathy. Systemic glucocorticoids were initiated with improvement in her dyspnea and skin lesions. Over the next several years, she did not tolerate weaning attempts and experienced frequent flares requiring escalating doses of methotrexate and glucocorticoids that led to significant side effects to include diabetes, obesity and bilateral hip avascular necrosis. Ultimately, her dyspnea and cough resolved on methotrexate and her skin lesions improved with a combination of intralesional triamcinolone injections and hydroxychloroquine with successful weaning off of her steroids. However, in subsequent visits, she reported swelling in her jaw along with a dry mouth with no recurrent skin lesions or dyspnea. On exam, enlargement of the parotid and lacrimal glands was recognized as Mikulicz syndrome. As a result, adalimumab was initiated with resolution of her symptoms. DISCUSSION: Mikulicz syndrome is a rare presentation of sarcoidosis, occurring in only 2% of patients with systemic sarcoidosis. It can easily be overlooked or attributed to a different disease process such as Sjogren’s syndrome, IgG4 related disease, lymphoma, vasculitis and HIV. However, it has been associated with significant symptoms affecting quality of life and most importantly represents active disease that requires consideration of treatment. In this case, the early recognition of Mikulicz syndrome led to appropriate treatment with a TNF-alpha antagonist that otherwise may not have been started as her skin and pulmonary sarcoid was relatively quiescent. CONCLUSIONS: Sarcoidosis can present with symptoms in nearly any organ system with significant impact on treatment course and quality of life. Providers who routinely treat sarcoid should be aware of the diverse manifestations of the disease, including the relatively rare presentations like Mikulicz syndrome as they may alter treatment strategy. Reference #1: Ungprasert, Patompong, Cynthia S. Crowson, and Eric L. Matteson. "Clinical characteristics of parotid gland sarcoidosis: a population based study.” JAMA otolaryngology--head & neck surgery 142.5 (2016): 503. Reference #2: Rao, Dinesh, et al. "A Case Report of Mikulicz Syndrome.” Journal of radiology case reports 11.7 (2017): 1. DISCLOSURES: No relevant relationships by Arthur Holtzclaw, source=Web Response No relevant relationships by John Sherner, source=Web Response

Exclusive abdominal lymphadenopathy as a rare presentation of sarcoidosis

Introduction: Sarcoidosis is a systemic granulomatous disease. The disease’s immunopathogenic mechanism is the activation of a cell mediated immune response. Lung is the most affected organ, but up to 30% of patients can have extrapulmonary manifestations.Case report: A 21-year-old male presented with a history of nausea, vomiting after meals, epigastric and periumbilical pain for approximately 6 months. Hyporexia and loss of 35 kilograms in 7 months were present, in addition to intermittent low nocturnal fever. His computed tomography (CT) scan (Figure 1) of the abdomen showed the presence of multiple, enlarged mesenteric lymph nodes with homogenous shape, measuring up to 2.0 cm in diameter, extending from the upper abdomen to the pelvis. Histopathologic findings of these lymph nodes showed a sarcoid appearance (Figure 2). Lymphoma,tuberculosis and other infections were excluded through immunohistochemical analysis. Treatment with prednisone was started initially at a dose of 40mg a day and after 5 weeks the patient was asymptomatic. One year and three months after the onset of treatment, corticosteroid dose reduction was attempted 3 times, with all of them leading to disease relapses. Treatment was optimized with 60mg of prednisone (for 2 weeks) plus 100mg of azathioprine daily.Discussion: The prevalence of gastrointestinal system involvement with clinical manifestations is 0,1 to 0,9%. However, the incidence of subclinical involvement can be higher. Enlarged lymph nodes are present in approximately 30% of cases. Treatment is indicated in sarcoidosis for patients with damage organ function or symptomatic disease and the first-line therapy is corticosteroids

Rare presentation of sarcoidosis: Multimodal imaging diagnosis of cardiac involvement

Rare presentation of sarcoidosis: Multimodal imaging diagnosis of cardiac involvement

A rare presentation of sarcoidosis with nasal bone involvement.

Background:Sarcoidosis is a multisystem granulomatous inflammatory disease that is induced by infectious or noninfectious environmental antigens in a genetically susceptible host. Tuberculosis and sarcoidosis are two diseases with similar clinical and pathologic findings. The link between these two diseases has been extensively studied.Objective:Herein we describe a case of sarcoidosis associated with tuberculosis, treated for tuberculosis, and, 1 year, later presented with a nasal dorsal lump and skin lesions on the extremities.Methods:Case report with clinical description.Results:Our patient had a history of skin and cervical lymphadenopathy symptoms 1 year earlier and was treated with antituberculosis drugs in an outer medical center. Therapy had cured cervical lymphadenopathies, with no improvement in skin lesions. On appearance of the nasal dorsal lump, she presented to our outpatient clinic. We retrieved the previous specimens of the patient, which revealed coexistence of necrotizing granulomas with non-necrotizing granulomas, which was strongly indicative of the coexistence of tuberculosis and sarcoidosis. Radiologic, histopathologic, and microbiologic investigation revealed the diagnosis of sarcoidosis with nasal, cutaneous, and pulmonary involvement. Treatment with prednisolone and hydroxychloroquine resulted in dramatic improvement of nasal bone, pulmonary, and skin lesions within 2 weeks.Conclusion:The clinical presentation of sarcoidosis can be complex, and the differential diagnosis from tuberculosis can be challenging. Atypical clinical pictures also can cause delays in diagnosis and proper management. In patients with granulomatous lesions that are unresponsive to antituberculosis therapy, physicians must be alerted to the possibility of coexistent sarcoidosis.

The Heerfordt-Waldenström Syndrome as an Initial Presentation of Sarcoidosis

Sarcoidosis is a granulomatous disease of unclear etiology, which commonly presents with cough, dyspnea, chest pain, fever, weight loss, arthralgias, and erythema nodosum. Heerfordt-Waldenström syndrome, a rare presentation of sarcoidosis, is characterized by the presence of parotid gland enlargement, facial palsy, anterior uveitis, and fever. Here we present a case of a 59-year-old nonsmoking African American woman who presented with 3 days of progressively worsening left facial droop, difficulty swallowing, and blurred vision. Over the prior 4 months, she had had a productive cough, fevers, night sweats, and an unintentional 30-pound weight loss. Physical examination revealed a left facial droop involving the forehead, cheek, and chin with an inability to close the left eyelid. Her serum angiotensin-converting enzyme level was twice the upper limit of normal. Prominent hilar markings were identified on chest x-ray, but no focal opacity was seen. Fine-needle aspiration of a preauricular lymph node revealed noncaseating granulomas consistent with granulomatous lymphangitis. The patient was given a diagnosis of Heerfordt-Waldenström syndrome, or uveoparotid fever. Treatment with a high-dose steroid improved her parotid gland enlargement, facial palsy, and anterior uveitis.

Case series and literature review of multiple nodular sarcoidosis

BackgroundNodular lung disease is a rare presentation of sarcoidosis. Radiologically it can present as multiple pulmonary masses or solitary lung nodule.Case presentationWe report three cases of nodular sarcoidosis in young females of Asian origin who had initially presented with dry cough and worsening dyspnea non-responsive to initially administered antibiotics. Pulmonary nodules were discovered upon radiographic imaging in all three cases which raised concern for the possibility of neoplastic processes. Subsequent biopsies revealed granulomatous inflammation indicative of sarcoidosis. All cases responded very well to systemic corticosteroids.ConclusionSarcoidosis may present as nodular infiltrates which alerts the treating physician to other neoplastic and infectious diseases of the lungs. Appropriate workup may reveal the true nature of this disease and hence, simplify treatment.

A rare presentation of sarcoidosis, back pain and spondylolisthesis

A 48-year old man presented with back pain that was resistant to treatment. An MR scan showed spondylolisthesis at L4-5 and narrowing of the exit foraminae. He had a posterior fusion which did not relieve his symptoms. He continued to have back pain and developed subcutaneous nodules in both forearms. Biopsy from the skin revealed cutaneous sarcoidosis, and one from the lumbar spine showed sarcoidosis granuloma between the bone trabeculae. A CT scan of the abdomen and chest revealed axillary lymphadenopathy, mediastinal enlarged nodes, apical nodular nodes and splenomegaly. The patient was started on large doses of methotrexate and steroids. His angiotensin-converting enzyme and calcium levels returned to normal and the back pain resolved.

A Case of Pseudoalveolar Sarcoidosis with Unilateral Pulmonary Infiltration

A sarcoidosis is a multisystemic granulomatous disorder that has a predilection for pulmonary involvement, and the common radiological findings for the disease are bilateral nodular or reticulonodular patterns. Pseudoalveolar sarcoidosis is a rare presentation of sarcoidosis. The radiological finding is an alveolar pattern that involves or compresses the alveoli by clustered interstitial granuloma. A 58-year-old man was admitted due to incidental findings of a unilateral consolidative lesion as seen on chest radiography. A chest computed tomography (CT) examination showed multiple bronchoalveolar consolidations that were suspicious of a malignancy. However, a percutaneous needle biopsy revealed non-caseating granuloma with an asteroid body that was compatible with sarcoidosis. After one month, the consolidative lesions improved without any treatment. (Tuberc Respir Dis 2008;64:149-152)

A rare presentation of sarcoidosis as a polypoid endobronchial structure

Summary Sarcoidosis is a multisystem disease of unknown etiology that predominantly affects intrathoracic lymph nodes and lungs. Although the diagnosis of sarcoidosis in some cases may be established on the ground of the clinical and radiological pattern, the American Thoracic Society in the 1999 statement emphasized that the diagnosis of sarcoidosis requires “histological demonstration of no necrotizing granulomas” [Hunninghake GU, Costabel U, Ando M et al. Statement on sarcoidosis. Am J Respir Crit Care Med 1999; 160 : 736–55]. We present the case of a very rare presentation of sarcoidosis as a polypoid endobronchial lesion coexisting with lymph node involvement. Diagnostics of sarcoidosis was confirmed in the histological examination of the specimens both from the lymph node and the polypoid lesion.

PSEUDOTUMOROUS RENAL INVOLVEMENT OF SARCOIDOSIS

Sarcoidosis, known mainly for pulmonary findings, can present with abdominal disease. Abdominal involvement most commonly affects the liver and spleen. Microscopic renal involvement is common but visible disease is unusual. Sarcoidosis of the kidney can cause nephrocalcinosis, nephrolithiasis, lesions or hydronephrosis. 1 Renal sarcoidosis is usually not visible on imaging and rarely presents as noncalcified renal masses. We report on a rare presentation of sarcoidosis with incidental bilateral noncalcified renal masses in the absence of renal dysfunction. CASE REPORT A 40-year-old black woman presented with right back and upper abdominal pain. She had a history of hypertension, cholelithiasis and a recent history of hematuria and urinary tract infection with suspected pyelonephritis. During the evaluation for source of infection, hilar adenopathy was discovered on a chest radiograph. Contrast enhanced abdominal computerized tomography (CT) revealed bilateral, multiple, wedge shaped renal masses from 2.0 to 4.6 cm. (fig. 1). The lesions were homogeneously hypodense and averaged 90 HU compared to adjacent parenchyma, which measured 205 HU. The lesions measured 29 HU on nonenhanced images and were well demarcated. There was no contour deformity of the renal margins. The kidneys were normal in size, and renal vasculature was unremarkable. Additional CT findings were retroperitoneal adenopathy, small hypodense hepatic lesions, gallstones and uterine leiomyomata. Both kidneys appeared normal on abdominal ultrasound for biopsy (fig. 2). No focal renal masses or contour deformities were identified. The cortical echo texture was homogeneous without focal areas of abnormality. After repeat contrast enhanced CT documented the persistence of the renal lesions, biopsy of the right renal lower pole, which had been the most severely affected region on CT, was performed. Pathological evaluation of the samples demonstrated noncaseating granulomas consistent with sarcoidosis. DISCUSSION