INTRODUCTION: Alveolar hemorrhage leading to respiratory failure is uncommon. Various etiologies have been reported, including systemic lupus erythematous (SLE), which generally presents as pulmonary-renal syndrome. We report a case of SLE with massive hemoptysis and acute renal failure(ARF), successfully treated with plasmapheresis.
CASE PRESENTATION: 65-years-old woman with history of hypertension and positive lupus anticoagulant presented with massive hemoptysis and respiratory failure. Patient denied chest pain, fever, recent upper respiratory symptoms, gastrointestinal or urinary problems. No toxic habits or travel history. She has two children with no history of abortions.On examination patient was in respiratory distress and hypoxemic with a SaO2 90% on 100% non rebreather mask. She was afebrile with diffuse crepitations on the right lung. Rest of examination was normal including blood pressure. Laboratory results showed hemolytic anemia (Hb 18 g/dl- baseline was 34 five months ago) ARF (creatinine 7.2 mg/dl -baseline 1.1 five months ago). Chest roentgenogram and computed chest tomogram revealed diffuse bilateral infiltrates predominantly on the right side. Perfusion scan and echocardiogram were normal.Serology was positive for ANA, Ds DNA. Antineutrophil cytoplasmic antibodies (cANCA), anti-glomerular basement membrane (anti-GBM) antibodies and cardiolipin antibodies were negative. The patient was intubated and fiberoptic bronchoscopy showed diffuse oozing of blood from all right lung segments. Hemosiderin-laden macrophages were seen in the BAL. A diagnosis of SLE with diffuse alveolar hemorrhage and ARF was entertained and patient was given pulse steroids and cyclophosphamide with some improvement in infiltrates and renal insufficiency and resolution of hemoptysis. Five days later she had recurrent hemoptysis with new left lung infiltrates and hypoxemia despite the use of continuous systemic corticosteroids. Three cycles of plasmapheresis were given with resolution of hemoptysis and infiltrates. Patient was successfully extubated. Kidney biopsy was not performed due to the critical state of the patient.Subsequently she had a prolonged hospital course complicated with nosocomial pneumonia, sepsis and multiorgan failure and finally she expired after three months of hospitalization.
DISCUSSIONS: Diffuse alveolar hemorrhage leading to respiratory failure is uncommon. Various etiologies have been reported, including infections, inhaled toxins, coagulation disorders, catastrophic antiphospholipid syndrome, Goodpasture's syndrome, microscopic polyangiitis and Wegener's granulomatosis, as well as various types of collagenosis, such as scleroderma and SLE.Pulmonary-renal syndrome is characterized by the occurrence of both alveolar hemorrhage and glomerular-nephritis and is frequently associated with the presence of antineutrophil cytoplasmic antibodies or anti-GBM antibodies. The type of injury, (alveolar, glomerular or both) determines the evolution and prognosis of the syndrome.A diagnosis of alveolar hemorrhage is made with the findings of hemoptysis, new alveolar infiltrate, anemia and the presence of blood or hemosiderin-laden macrophages in BAL like in our patient. In SLE patients, diffuse alveolar hemorrhage is uncommon, occurring in only 2% of cases, and is often associated with mortality rates between 70-90%. Recurrent hemoptysis is seen in 10% of the cases. Concomitant renal involvement is also observed. Histological studies have shown that, in about 70% of lung biopsies, there are little inflammatory activity and a predominance of hemorrhagic characteristics, whereas in the remaining 30%, a neutrophilic capillaritis or diffuse alveolar damage is seen. Pulse steroids followed by high dose steroids with cyclophosphamide have been shown to control pulmonary hemorrhage. In case series, plasmapheresis had been used for recurrent hemoptysis although no survival benefit has been shown. Tacrolimus and immunoglobulin can be tried in refractory hemoptysis.
CONCLUSION: Diffuse alveolar hemorrhage is a life-threatening manifestation of SLE. An active diagnostic workup and aggressive immunosuppressive treatment are the cornerstones of the management. Early detection and active treatment of secondary infections are obligatory. Plasmapheresis needs to be considered in cases with refractory hemoptysis.
DISCLOSURE: Praveen Rudraraju, None.