Shrinking lung syndrome in systemic lupus erythematosus-scleroderma overlap

• Main Authors: Vivek S Guleria, Pradeep K Singh, Puneet Saxena, Shankar Subramanian
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2014-01-01
• Series: Lung India
• Subjects: Scleroderma; scleroderma shrinking lung syndrome; systemic lupus erythematosus
• Online Access: http://www.lungindia.com/article.asp?issn=0970-2113;year=2014;volume=31;issue=4;spage=407;epage=409;aulast=Guleria

Shrinking lung syndrome (SLS) is a infrequently reported manifestation of systemic lupus erythematosus (SLE). Reported prevalence of SLS is about 0.5% in SLE patients. Pathogenesis is not fully understood and different therapeutic modalities have been employed with variable results, as only 77 cases of SLS have been documented in literature. SLS in SLE-Scleroderma overlap has not been reported yet. We report a patient of SLE - scleroderma overlap presenting with dyspnea, intermittent orthopnea and pleuritic chest pain. Evaluation revealed elevated hemidiaphragms and severe restrictive defect. She was eventually diagnosed as a case of SLS. This case report is a reminder to the medical fraternity that SLS although a rare complication must be thought of in the special subset of patients of SLE having respiratory symptoms.