Dramatic response to plasma exchange in systemic lupus erythematosus with acute complications: Report of two cases
A 28-year-old female presented with history of shortness of breath, cough with blood stained sputum and oliguria for 1 day. There was history of pedal edema, multiple joint pains associated with swelling for 10 days, with history of oral ulcers off and on and polymenorragia for 6-8 months. There was also history of untreated mild to moderate hypertension for 2 years. She was admitted inntensive care unit (ICU) for ventilator support. On examination, Jugular Venous Pressure (JVP) was raised with severe pallor and pedal edema. Auscultation of chest revealed fine crackles all over the chest. Abdominal examination showed hepatomegaly. X-ray and computed tomography (CT) scan of chest showed diffuse alveolar opacities in bilateral upper and middle lobes of lungs. Initial urea and creatinine values were 89 mg/dl and 2.2 mg/dl respectively. Serology revealed positive ANA and anti double strandard DNA antibodies. Patient was diagnosed as SLE with acute flare up in the form of diffuse alveolar hemorrhage and renal failure. Patient was intubated and put on a ventilator, started with pulse methylprednisolone therapy and injection cyclophosphamide. Three units of packed red cells were transfused for severe anemia (Hb-4.7 g/dl). As there was no improvement after 48 h, the treating physician planned plasma exchange for the patient. The patient was assessed for the procedure by transfusion medicine team and the patient was shifted to department of transfusion medicine on ventilatory support. One plasma volume of patient was exchanged with normal saline and 4% human serum albumin as replacement fluid. No procedural complications were observed and he was shifted back to ICU. After 24 h of the procedure, patient was successfully weaned off the ventilator and extubated with no fresh episodes of hemoptysis. However, urine output was still low with hematuria. A total of four plasma exchange cycles were done for the patient on alternate days and one plasma volume was exchanged each time. After the 2nd procedure, there was an improvement in urine output without any need for renal replacement therapy. After the 3rd procedure, urine output was normal without hematuria, with no fresh episodes of hemoptysis and a repeat X-ray showed clearing of hemorrhagic opacities from lungs. Patient was discharged after the 4th procedure, though azotemia persisted. Her total duration of hospital stay was 14 days with ICU stay of 10 days.