A79: Pulmonary Hemorrhage in Pediatric Systemic Lupus Erythematous: Clinical Course and Outcomes

Abstract

Background/Purpose: Pulmonary hemorrhage (PH) is a rare and devastating manifestation of SLE, occurring in 1–5% of adult cohorts with mortality rates ranging from 70%‐90%. The majority of PH reported in SLE refers to the adult population, and there is a paucity of data in pediatrics. Therefore, we describe our experience with PH in pediatric SLE, outlining disease presentation, treatment, and outcomes. Methods: We reviewed records of pediatric SLE patients who presented with pulmonary involvement diagnosed between January 2000 and August 2010. Inclusion criteria included > 4 SLE classification criteria and PH based on pulmonary evaluation by BAL, lung biopsy and/or characteristic imaging with worsening anemia. We describe our pediatric cohort, presenting symptoms, diagnostic modalities, various interventions and overall outcomes. Results: Seven of 401 (1.7%) SLE patients met inclusion criteria for PH. Patients were predominantly male (71.4%) and Hispanic (57.1%). Median age of PH presentation was 13.5 years (range 2.0–15.2). Episodes were primarily within the first 3 months of SLE diagnosis (71.4%) and were a presenting manifestation for SLE in 28.6% of our patients. All patients presented with a new cough, dyspnea, hypoxemia, anemia and abnormal CXR. Hemoptysis was present in 28.6%. Four out of 6 children had chest CT findings with diffuse ground glass opacities (66.6%) and 3 with nodules (50%). Additional non‐pulmonary features included fever (71.4%), renal disease (57.1%), cytopenias (71.4%), cardiac disease (50.0%), and positive antiphospholipid antibodies (85.7%). Eighty‐five percent required supplemental oxygen and 57.1% required mechanical ventilation. Hemosiderin‐laden macrophages without infection were observed in 3 BAL's. Out of the two lung biopsies performed, both revealed diffuse hemorrhage and one demonstrated immunoglobulin and complement deposition. All were treated with pulse doses of methylprednisolone infusions. Other therapeutic interventions included cyclophosphamide (42.8%), plasma exchange (42.8%), rituximab (85.7%) and IVIG (14.2%). Survival rate was 87.5%. All patients were maintained on corticosteroids and 66.6% received monthly cyclophosphamide infusions. Subsequent outpatient therapies included IVIG (33.3%), azathioprine (16.6%) or rituximab (16.6%). No patients had recurrences of PH. No survivors required supplemental oxygen nor developed interstitial lung disease (median follow‐up 2.6 years, range 1.5–5.0). Conclusion: In our review of pSLE patients with PH, the prevalence of this complication approximated that of adult SLE cohorts. However, in contrast to the adult population, PH presented earlier in the course of SLE in children. Additionally, outcomes were favorable with initiation of aggressive, multi‐modal immunosuppressive therapy with no recurrences. The mortality rate was much lower than described in adult cohorts.

Management of massive diffuse alveolar hemorrhage in a child with systemic lupus erythematosus

“…One type of pulmonary hemorrhage, DAH, is a rare and life-threatening complication of SLE associated with high mortality [ 5 ]. DAH may present in the early course of SLE in children [ 1 ] and is associated with high disease activity [ 6 ]. Bleeding into the alveolar spaces characterizes the syndrome of DAH and is due to disruption of the alveolar-capillary basement membrane caused by injury or inflammation of the arterioles, venules, or alveolar septal (alveolar wall or interstitial) capillaries.…”

“…These interventions would not have been successful if medical treatment against SLE was not initiated as soon as possible. The main therapy for DAH with SLE in children and adults is high-dose methylprednisolone [ 1 ] combined with cyclophosphamide [ 6 ] and plasmapheresis [ 5 ]. The other therapeutic options are rituximab, IVIG, and mycophenolate mofetil [ 6 ].…”

“…A recent report of seven cases of pediatric systemic lupus erythematosus (SLE) with pulmonary hemorrhage had a survival rate of 87.5% [ 1 ]; however, the survival rate for diffuse alveolar hemorrhage (DAH) is extremely low when it leads to acute catastrophic hemoptysis with impairment in oxygenation, ventilation, or hemodynamic instability. In adults, high Acute Physiology and Chronic Health Evaluation II scores, requirement of mechanical ventilation, renal failure, and thrombocytopenia are associated with higher mortality in DAH with SLE [ 2 ].…”