Case Report: Sarcoidosis with azygos vein enlargement mimicking metastatic cancer

Sarcoidosis is a systemic disease with heterogeneous clinical manifestations that is characterized histologically by the presence of noncaseating granulomas in the affected organs. It can be a diagnostic challenge, especially when mimicking malignancy or fungal infections. Previous case reports of sarcoidosis presenting with multiple masses are highly suggestive of infectious or malignant etiology. In this case, our patient presented with enlarged lymph node and was found to have innumerable nodules in the mediastinum, lungs, and liver. Azygos vein enlargement was also seen on radiological imaging, and malignancy was highly suspected; hence, an extensive workup was conducted, including laboratory, radiology and biopsy evaluation, which were diagnostic of sarcoidosis. Our case showed the importance of correlation of the history, physical examination, radiological and histopathologic studies in confirming the diagnosis and the need to rule out other serious infections and malignancies, especially with azygous vein enlargement, which can sometimes be missed in chest radiograph.

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Sarcoidosis

10.2310/im.1209 ◽

2020 ◽

Author(s):

Robert P Baughman ◽

Mary Beth Scholand

Keyword(s):

Cardiac Sarcoidosis ◽

Systemic Disease ◽

Clinical Manifestations ◽

Host Factors ◽

Resonance Imaging ◽

Noncaseating Granuloma ◽

Symptomatic Disease ◽

Contrast Enhanced ◽

Löfgren Syndrome ◽

Noncaseating Granulomas

Sarcoidosis is a systemic disease characterized by the presence of noncaseating granulomas, which accumulate in affected organs. The incidence, organ involvement, and disease severity depend on environmental exposures and host factors. The cause of sarcoidosis remains unknown. Any organ can be affected; however, involvement of the lung, heart, and nervous system contributes most to morbidity and mortality. This review discusses the epidemiology, etiology, genetics, pathogenesis, diagnosis (including clinical manifestations), differential diagnosis, management, complications, and prognosis of sarcoidosis. Figures depict the pathogenesis of sarcoidosis, radiographic stages of sarcoidosis, contrast-enhanced magnetic resonance imaging of a patient with neurosarcoidosis, noncaseating granuloma from a tissue biopsy of a patient with sarcoidosis, various manifestations of sarcoidosis, approach to the use of anti-inflammatory therapy for sarcoidosis, and an algorithm for symptomatic disease. This review contains 7 highly rendered figures, 7 tables, and 127 references Keywords: cardiac sarcoidosis, hilar lymphadenopathy, Löfgren syndrome, neurosarcoidosis, noncaseating granuloma, sarcoidosis, uveitis

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PS01.018: PERSISTENT DYSPHAGIA AFTER FUNDOPLICATION WITH MESH PLASTY –MISSED OR MISTAKEN –A CASE REPORT

Diseases of the Esophagus ◽

10.1093/dote/doy089.ps01.018 ◽

2018 ◽

Vol 31 (Supplement_1) ◽

pp. 55-55

Author(s):

Servarayan Chandramohan ◽

Visvarath Varadharajan ◽

Madeshwaran Chinnathambi ◽

Kanagavel Manickavasagam ◽

Abishai Jebaraj ◽

...

Keyword(s):

Skin Biopsy ◽

Mesh Repair ◽

Conflicts Of Interest ◽

Case Reports ◽

Systemic Disease ◽

Transhiatal Esophagectomy ◽

High Resolution Manometry ◽

Failed Fundoplication ◽

Rule Out

Abstract Background Scleroderma esophagus is a rare entity. Only few case reports of esophagectomy were done and reported for this condition. We are presenting this rare case of failed fundoplication and mesh repair with a diagnosis of GERD and hiatus hernia, which was found later on due to Scleroderma with Esophageal involvement. Methods 58 year old female admitted with dysphagia following laproscopic fundoplication with mesh repair of crura with a diagnosis of GERD and hiatus hernia.She presented with persistent vomiting and loss of weight.On evaluation, her Upper GI scopy revealed dilated esophagus with sluggish peristalisis. Since the patient had tightness of skin over the distal extremities, face and fish mouth appearance with thinning of nail, Skin biopsy was taken. The skin biopsy was reported to be scleroderma.The esophageal manometry demonstrated failed esophageal peristalisis with high normal LES pressure due to tight fundal wrap.The patient was treated with mesh remova, Transhiatal esophagectomy with gastric pull-up and cervical Anastomosis.Post operatively the patient was treated with hydrocholoroquine and predinisolone. Results The patient is free of dysphagia and is on regular follow up. Conclusion In case of failure, detailed evaluation including High resolution manometry (MII HRM) has to be done before doing laparoscopic fundoplication for GERD has to rule out uncommon and rare disorders of esophagus. Detailed clinical examination in GERD patients has to be done to rule out systemic disease like scleroderma.In case of failed fundoplication for GERD, patients have to investigated for the failure.So patients with incapacitating esophageal neuromotor disease, a more radical approach in the form of esophagectomy may be safer and more reliable than attempting another procedure and risk another failure. Disclosure All authors have declared no conflicts of interest.

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Sarcoidosis

10.2310/fm.1209 ◽

2020 ◽

Author(s):

Robert P Baughman ◽

Mary Beth Scholand

Keyword(s):

Cardiac Sarcoidosis ◽

Systemic Disease ◽

Clinical Manifestations ◽

Host Factors ◽

Resonance Imaging ◽

Noncaseating Granuloma ◽

Symptomatic Disease ◽

Contrast Enhanced ◽

Löfgren Syndrome ◽

Noncaseating Granulomas

Sarcoidosis is a systemic disease characterized by the presence of noncaseating granulomas, which accumulate in affected organs. The incidence, organ involvement, and disease severity depend on environmental exposures and host factors. The cause of sarcoidosis remains unknown. Any organ can be affected; however, involvement of the lung, heart, and nervous system contributes most to morbidity and mortality. This review discusses the epidemiology, etiology, genetics, pathogenesis, diagnosis (including clinical manifestations), differential diagnosis, management, complications, and prognosis of sarcoidosis. Figures depict the pathogenesis of sarcoidosis, radiographic stages of sarcoidosis, contrast-enhanced magnetic resonance imaging of a patient with neurosarcoidosis, noncaseating granuloma from a tissue biopsy of a patient with sarcoidosis, various manifestations of sarcoidosis, approach to the use of anti-inflammatory therapy for sarcoidosis, and an algorithm for symptomatic disease. This review contains 7 highly rendered figures, 3 tables, and 126 references Keywords: cardiac sarcoidosis, hilar lymphadenopathy, Löfgren syndrome, neurosarcoidosis, noncaseating granuloma, sarcoidosis, uveitis

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Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice—Case Report and Literature Review

Case Reports in Rheumatology ◽

10.1155/2018/9602373 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5

Author(s):

Pragya Shrestha ◽

Brian Le ◽

Brent Wagner ◽

William Pompella ◽

Paras Karmacharya

Keyword(s):

Sclerosing Cholangitis ◽

Plasma Cells ◽

Systemic Disease ◽

Clinical Manifestations ◽

Disease Process ◽

High Serum ◽

Diagnostic Challenge ◽

Obliterative Phlebitis ◽

Immunoglobulin G4 ◽

Serum Igg4

IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC). IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs. The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis. High serum IgG4 levels, characteristic radiological (e.g., sausage-shaped pancreas or periaortitis) or biopsy findings (high percentage of IgG4+ plasma cells, lymphoplasmacytic infiltrate, storiform fibrosis, or obliterative phlebitis) in the setting of these features is diagnostic of this disease process. However, isolated IgG4-SC might be a diagnostic challenge, and the distinction is important as management of this disorder is vastly different from other causes of cholangitis such as PSC. Systemic corticosteroid therapy is the mainstay of therapy.

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Granulomatosis with Polyangiitis in Adolescence: Two Distinct Presentations

Case Reports in Rheumatology ◽

10.1155/2021/6642910 ◽

2021 ◽

Vol 2021 ◽

pp. 1-7

Author(s):

Rafael Figueiredo ◽

Inês Pires Duro ◽

António Marinho ◽

Conceição Mota ◽

Margarida Guedes ◽

...

Keyword(s):

Granulomatosis With Polyangiitis ◽

Case Reports ◽

Systemic Disease ◽

Young Patients ◽

Clinical Manifestations ◽

Splenic Infarction ◽

Bronchial Biopsy ◽

Vein Thrombosis ◽

Individualized Approach ◽

Deep Vein

Introduction. Granulomatosis with polyangiitis (GPA) is a rare disease in pediatric age. We report two cases with distinct presentations. Case Reports. A seventeen-year-old male with prolonged febrile syndrome, cough, and constitutional symptoms. CT-scan showed cavitated lesions of the lung and bronchial biopsy a necrotizing inflammatory process. The remaining investigation revealed hematoproteinuria and positive C-ANCA and anti-PR3. Complications: Bilateral acute pulmonary thromboembolism, splenic infarction, and extensive popliteal and superficial femoral deep vein thrombosis. He was treated with corticosteroids, immunoglobulin, rituximab, and anticoagulation. Rituximab was maintained every six months during the first two years. Control angio-CT was performed with almost complete resolution of previous findings. In a twelve-year-old female with inflammatory signs of the limbs, investigation showed myositis of the thigh and tenosynovitis of the wrist, normocytic normochromic anemia (Hg 9.4 g/dL), mild elevation of inflammatory markers, and high creatine kinase. During hospitalization, she presented an extensive alveolar hemorrhage associated with severe anemia and positive C-ANCA and anti-PR3. Clinical deterioration prompted intravenous methylprednisolone pulses and plasmapheresis. Induction therapy with rituximab and prednisolone showed good results. Rituximab was maintained every six months, for 18 months, with gradual tapering of corticoids. Discussion. GPA is a systemic disease with variable clinical presentation and severity. Pediatric patients have similar clinical manifestations to adults but different frequencies of organ involvement; constitutional symptoms are also more common. We highlight the different presentation of these two cases, as well as the need for an individualized approach. Rituximab has been used for both induction-remission and maintenance therapy, with good results, particularly in young patients.

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Lymphadenitis “As Rosary beads” - a diagnostic challenge

ARS MEDICA Revista de Ciencias Médicas ◽

10.11565/arsmed.v44i3.1564 ◽

2019 ◽

Vol 44 (3) ◽

pp. 20-23

Author(s):

Vitorino Modesto Dos Santos ◽

Micheline Silva Abreu De Azevedo ◽

Katia Rejane Marques Brito ◽

Larissa Almondes Da Luz ◽

Kayursula Dantas Ribeiro

Keyword(s):

Case Reports ◽

Clinical Manifestations ◽

Definitive Diagnosis ◽

Medical Attention ◽

Diagnostic Challenge ◽

Regional Lymph Nodes ◽

Primary Health ◽

Primary Focus ◽

The Right ◽

High Index Of Suspicion

Introduction: nodular lymphangitis (NL) may be a manifestation of various infectious and non-infectious conditions, characterised by subcutaneous inflammatory nodules that extend from the site of the primary focus to the regional lymph nodes. NL usually constitutes a challenge in primary health care, and histopathological and microbiological evaluations are necessary to establish the definitive diagnosis. Methods: We report a classic case of NL that developed in the upper limb of a middle-aged man after trauma on the right thumb. The objective: was to emphasise some aspects of the differential diagnosis in a patient with NL and to reduce the rate of misdiagnosis in the scenery of initial medical attention. Results: the clinical manifestations were non-specific, but the microbiological study revealed typical characteristics of sporotrichosis. The treatment with itraconazole (200 mg daily) for six months was successful. Conclusion: NL constitutes a challenging condition and the early diagnosis depends on a high index of suspicion. Case reports may reduce late diagnoses with unfavourable results.

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Hydrocephalus as the first presenting symptom of neurosarcoidosis in two patients: a diagnosis more forthcoming in the context of systemic disease

BMJ Case Reports ◽

10.1136/bcr-2019-229903 ◽

2019 ◽

Vol 12 (8) ◽

pp. e229903 ◽

Cited By ~ 2

Author(s):

Anna McKeever ◽

Amanda Cox ◽

Matthew Garnett ◽

Nicholas G Cunniffe

Keyword(s):

Physical Disability ◽

Systemic Disease ◽

Clinical Manifestations ◽

Neurological Involvement ◽

High Morbidity ◽

Diagnostic Challenge ◽

Multisystem Disorder ◽

Central Nervous System Inflammation ◽

Surgical Interventions ◽

Granulomatous Diseases

Sarcoidosis is a multisystem disorder, characterised histologically by the presence of non-caseating epithelioid granulomas with exclusion of other granulomatous diseases. While the lungs and lymph nodes are affected in 90%, approximately 5% of patients have neurological involvement. The clinical manifestations of neurosarcoidosis (NS) are diverse, making diagnosis especially difficult in patients without known systemic disease. Hydrocephalus occurs in only 9% of patients with NS and although uncommon, is an important manifestation because it is associated with high morbidity and mortality. We report two cases of NS presenting with hydrocephalus, one as the first presentation of sarcoidosis and one in a patient with known multisystem sarcoidosis. The patient without systemic sarcoidosis posed the greater diagnostic challenge and followed a protracted course with multiple surgical interventions, progression of central nervous system inflammation and significant physical disability.

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Neurosarcoidosis: guidance for the general neurologist

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2012000400014 ◽

2012 ◽

Vol 70 (4) ◽

pp. 293-299 ◽

Cited By ~ 8

Author(s):

Lívia Almeida Dutra ◽

Pedro Braga-Neto ◽

Ricardo Araújo Oliveira ◽

José Luiz Pedroso ◽

Agessandro Abrahão ◽

...

Keyword(s):

Systemic Disease ◽

Symptom Control ◽

Immunosuppressive Agents ◽

Clinical Manifestations ◽

Clinical History ◽

Tnf Alpha ◽

Pathology Laboratory ◽

High Doses ◽

Diagnostic Work ◽

Noncaseating Granulomas

Neurosarcoidosis (NS) more commonly occurs in the setting of systemic disease. The diagnosis is based on a clinical history suggestive of NS, presence of noncaseating granulomas, and supportive evidence of sarcoid pathology, laboratory, and imaging studies. NS could involve any part of the nervous system and often demands high doses of steroids for symptom control. It presents low response to isolated steroids administration and frequently requires immunosuppressive agents. In NS, lymphocytes are polarized toward an excessive Th1 response, leading to overproduction of TNF-alpha and INF-gama, as well as lL-2 and IL-15. Infliximab, a chimeric monoclonal antibody that neutralizes the biological activity of TNF-alpha, is a new option in the NS treatment. We revised pathophysiology, clinical manifestations, diagnostic work up, and treatment of NS as guidance for the general neurologist.

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Cat Scratch Disease

Pediatrics in Review ◽

10.1542/pir.15.9.348 ◽

1994 ◽

Vol 15 (9) ◽

pp. 348-353 ◽

Cited By ~ 1

Author(s):

Joel D. Klein

Keyword(s):

Erythema Nodosum ◽

Close Contact ◽

Case Reports ◽

Systemic Disease ◽

Clinical Manifestations ◽

Central Nervous System Involvement ◽

The United States ◽

Ocular Involvement ◽

Cat Scratch Disease ◽

Etiologic Agents

Introduction Cat scratch disease (CSD), a regional lymphadenitis affecting those lymph nodes draining dermal and/or conjunctival sites of inoculation, probably is the most common cause of chronic lymphadenopathy among children and adolescents. Adults account for only 10% to 20% of cases. It usually presents as a benign, self-limited disease involving tender regional lymphadenopathy that frequently has been present for 3 weeks or longer. In most cases the infection is preceded by a cat scratch or other close contact with a cat (generally an immature one). This infection's association with cat scratches was described first by Robert Debre in 1931. Since then, the spectrum of CSD has been expanded to include the presence of various skin rashes, such as erythema nodosum and erythema multiforme, ocular involvement, and constitutional symptoms such as fatigue and fever. In addition, many case reports of CSD have included descriptions of atypical clinical manifestations such as central nervous system involvement, arthritis, osteomyelitis, and severe chronic systemic disease. Most recently, two possible etiologic agents of CSD have been identified: a fastidious, pleomorphic gram-negative bacillus, Afipia felis, and a rickettsial pathogen named Rochalimaea henselae. Epidemiology CSD appears to occur worldwide and is seen in all regions of the United States. Cases may be seen sporadically all year long, but it appears to have a seasonal preference.

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Gastric Sarcoidosis: A Rare Clinical Presentation

Case Reports in Gastrointestinal Medicine ◽

10.1155/2013/260704 ◽

2013 ◽

Vol 2013 ◽

pp. 1-3

Author(s):

Hemasri Tokala ◽

Karthik Polsani ◽

Jagadeesh K. Kalavakunta

Keyword(s):

Clinical Presentation ◽

Epigastric Pain ◽

Case Reports ◽

Systemic Disease ◽

Interesting Case ◽

Histological Evidence ◽

Gi Symptoms ◽

Oral Steroids ◽

History Of ◽

Noncaseating Granulomas

Gastrointestinal (GI) sarcoidosis is a very rare disease, which clinically presents along with systemic disease or as an isolated finding. Gastric sarcoidosis is the most common form of GI sarcoidosis. Symptomatic gastric sarcoidosis is rare and only few case reports have been described in the literature with well-documented histological evidence of noncaseating granulomas. We present an interesting case of gastric sarcoidosis in a 39-year-old Caucasian man with symptoms of epigastric pain and profound weight loss. His endoscopic gastric mucosal biopsies revealed noncaseating granulomas consistent with gastric sarcoidosis. Treatment with oral steroids alleviated his symptoms with no recurrence in 2 years. Gastric sarcoidosis should be considered in patients with history of sarcoidosis and GI symptoms.

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