Two cases of systemic lupus erythematosus (SLE) with acute lupus pneumonitis chracterized by the symptoms of high fever, severe dyspnea and hypoxemia were presented. Case 1 was a 64-years-old male with acute onset of SLE. By the initial treatment with prednisolone, SLE was well controlled, however, acute lupus pneumonitis did not improve and was fatal despite of pulse therapies with methylprednisolone and cyclophosphamide and plasma exchange. Case 2 was 33-years-old female who suffered from SLE and lupus nephritis for 9 years. She was admitted for worsening of lupus nephritis. Despite of initial treatment with prednisolone and pulse therapy with methylprednisolone, Iupus nephritis slowly progressed. Subsequently she was complicated with fatal acute lupus pneumonitis, pulse therapy with methylprednisolone, immunoadsorption and plasma exchange being ineffective.