The main aim of this study is to understand Secondary pulmonary alveolar proteinosis (PAP) associated with treatment is relatively frequent1,2. However, PAP associated with autoimmune diseases is extremely rare; we have found only 2 cases reported in the literature1,3.

We present a 34-year-old woman diagnosed with systemic lupus erythematosus (SLE) in 2001 based on rash malar, arthritis, leukopenia, positive antinuclear antibody, positive anti-DNA, and hypocomplementemia. She had received immunosuppressive drugs (methotrexate, belimumab, mycophenolate, and leflunomide).

She was admitted to a hospital in September 2014 for the study of respiratory insufficiency. An interstitial pattern could be observed in the thorax radiography and it was interpreted as lupus pneumonitis. She received treatment with methylprednisolone bolus and rituximab. The patient’s disease course declined and in December 2014 she was referred to our hospital to assess the need for lung transplant. Serological and hematological activity of SLE was discarded. Thoracic computerized tomography showed a crazy-paving pattern.

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