Sarcoidosis and Systemic Sclerosis: Strange Bedfellows

Coexistence of systemic sclerosis and sarcoidosis is rare. Both have predominant lung manifestations, each with distinctive features on computed tomography (CT) of the chest. We present herein a 52-year-old male with limited systemic sclerosis manifested primarily by sclerodactyly and subsequently b...

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Bibliographic Details
Published in:Case reports in rheumatology 2017-01, Vol.2017 (2017), p.1-4
Main Authors: Kirk, Shannon, Maken, Kanwaljeet, Lezcano, Sheila D., Sandhu, Vaneet K., Yu, Micah, Torralba, Karina D.
Format: Article
Language:English
Subjects:
Autoimmune diseases
Care and treatment
Case Report
Case reports
Case studies
Chemokines
Critical care
Cysts
Diagnosis
Dyspnea
Gene expression
Health risk assessment
Laboratories
Lung diseases
Lupus
Medicine
Pathogenesis
Patients
Pneumonia
Pulmonary fibrosis
Rheumatoid arthritis
Rheumatology
Sarcoidosis
Scleroderma
Scleroderma (Disease)
Systemic scleroderma
Systemic sclerosis
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Summary:Coexistence of systemic sclerosis and sarcoidosis is rare. Both have predominant lung manifestations, each with distinctive features on computed tomography (CT) of the chest. We present herein a 52-year-old male with limited systemic sclerosis manifested primarily by sclerodactyly and subsequently by shortness of breath. A series of CT scans of the chest were reviewed. Initial CT chest one year prior to sclerodactyly onset revealed bilateral hilar and right paratracheal, prevascular, and subcarinal adenopathy. Five-year follow-up demonstrated thin-walled cysts, mediastinal lymphadenopathy, and nonspecific nodules. Due to progression of dyspnea, follow-up CT chest after one year again demonstrated multiple cysts with peripheral nodularity and subpleural nodules, but no longer with hilar or mediastinal adenopathy. Diagnostic open lung biopsy was significant for noncaseating granulomas suggestive of sarcoidosis. This is the first known case of a patient with systemic sclerosis diagnosed with sarcoidosis through lung biopsy without radiographic evidence of hilar or mediastinal lymphadenopathy at the time of biopsy. A review of cases of concomitant sarcoidosis and systemic sclerosis is discussed, including the pathophysiology of each disease with shared pathways leading to the development of both conditions in one patient.
ISSN:2090-6889
2090-6897
DOI:10.1155/2017/7851652