Lupus shrinking lung syndrome with good response with corticosteroid and methotrexate: a case report

Shrinking lung syndrome (SLS) is a rare pleuropulmonary manifestation of systemic lupus erythematosus (SLE) characterized by diaphragmatic dysfunction and a restrictive pattern on pulmonary function testing with normal pulmonary parenchyma. The estimated prevalence ranges between 0.5 and 10%. Although the cause of SLS remains controversial, one possible mechanism is diaphragm`s myositis or myopathy resulting in its elevation and decreased function. Factors associated with SLS include greater disease duration, seropositivity for anti-RNP antibodies and a history of pleuritis. Clinically, patients present with progressive dyspnea. Although there is low level of evidence supporting the use of immunosuppressive therapy for SLS, glucocorticoids and immunosuppressive therapy (cyclophosphamide, mycophenolate and azathioprine) may improve both symptoms and pulmonary function. Noninvasive positive pressure ventilation may also be beneficial We describe the case of a patient with SLE who manifested SLS soon after the diagnosis and presented a good response with the treatment performed