Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary sarcoidosis is usually a chronic, insidious disease resulting from granuloma formation in the lung parenchyma. The epithelioid non-caseating granulomata of sarcoidosis are the result of a T-helper1-mediated immune reaction to an unknown self or foreign antigen. We describe the case of a patient with sarcoidosis and a coexistent common variable immunodeficiency who presented with rapidly progressive respiratory failure. This unusual presentation was followed by a complicated course with recurrent pneumoccocal infections, which could be explained by the coexistence of common variable immunodeficiency. Physicians should be alert to the possibility of sarcoidosis even when the clinical presentation is of acute respiratory distress syndrome (ARDS) since early treatment with steroids can be lifesaving. The detection of accompanying hypogammaglobulinemia is also crucial, as treatment with intravenous immunoglobulins (IVIG) together with steroids can improve the patient's outcome.
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PMID:Acute respiratory failure in a patient with sarcoidosis and immunodeficiency--an unusual presentation and a complicated course. 1513 81