Rituximab in the treatment of shrinking lung syndrome in systemic lupus erythematosus.

Association of Macrophage Activating Syndrome with Castleman’s Syndrome in Systemic Lupus Erythematosus

Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still's disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Ira...

Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide.

Shrinking lung syndrome (SLS) is an uncommon feature of systemic lupus erythematosus (SLE) characterized by dyspnea, pleuritic chest pain, diaphragmatic elevation, restrictive ventilatory defect and reduced respiratory muscle strength as measured by volitional tests. We report the case of a 28-year-old woman with overlapping features of SLE and Sjögren syndrome who developed severe SLS while re...

Oral contraceptive-related transverse sinus thrombosis as an initial manifestation of antiphospholipid syndrome in the absence of systemic lupus erythematosus

Anticardiolipin Antibodies in Juvenile Rheumatoid Arthritis and Systemic Lupus Erythematosus

Background: Antiphospholipid antibody syndrome (APS) can either occur as a primary syndrome or associated with other autoimmune diseases such as systemic lupus erythematosus (SLE). Anticardiolipin antibody (aCL) of IgG and/or IgM isotype in blood, measured by a standardized ELISA is the most acceptable laboratory criteria. APS IgG isotype, particularly IgG2 subclass is more strongly associated ...

PReS-FINAL-2300: Shrinking lung syndrome in an adolescent male with juvenile systemic lupus erythematosus

Introduction Pulmonary involvement occurs in up to 80% of Systemic Lupus Erythematosus (SLE) patients, with decreased diffusing capacity (DLCO) and interstitial lung disease being most common. Shrinking lung syndrome (SLS) is a rare complication, which consists in small lung volumes, elevation of the diaphragm and restrictive physiology without parenchymal or pleural involvement. Its pathogenes...