Open AccessShrinking lung syndrome in systemic lupus erythematosus-scleroderma overlap
Author(s) -
Vivek Singh Guleria,
Pradeep K Singh,
Puneet Saxena,
Shankar Subramanian
Publication year - 2014
Publication title -
lung india
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 25
eISSN - 0974-598X
pISSN - 0970-2113
DOI - 10.4103/0970-2113.142152
Subject(s) - medicine , scleroderma (fungus) , dermatology , overlap syndrome , systemic scleroderma , complication , systemic lupus erythematosus , lung , lupus erythematosus , rheumatology , pathology , immunology , dermatomyositis , disease , antibody , inoculation , copd
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.