We conclude that open lung biopsy is a useful tool in the management of acute respiratory failure when there is no clinical improvement after standard treatment, since it can lead to a specific diagnosis that requires distinct treatment, which probably lowers the mortality rate among such patients.

The association of systemic lupus erythematosus (SLE) with amyloidosis is exceptional. We present a 37-year-old patient who was diagnosed five months earlier for SLE. She developed an acute episode of chest pain, cough and dyspnoea. Hypoxemia and obstructive changes in respiratory tests were present. The chest X-ray was repeatedly normal. Open lung biopsy revealed lupus pneumonitis with positive stain for immunoglobulins and complement, bronchiolitis obliterans, and pulmonary amyloidosis.

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