Background Diffuse Alveolar hemorrhage (DAH) a rare life threatening manifestation of Systemic Lupus Erythematosus (SLE) with high mortality.1,2
Objectives To characterize the profile of SLE patients with DAH in our cohort.
Methods Medical charts of patients with SLE and DAH from 2008 to 2015 in our institute were reviewed. Clinical, laboratory, treatment, outcome details were noted. Statistical analysis carried out with SPSS software (version 21). Independent sample T test was done to compare survivors and non-survivors.
Results Nineteen (1.32%) of 1438 SLE patients had DAH. Their mean age was 24.2±7.8 years, mean duration of hospital stay 16.1±7.5 days, mean SLE disease activity index (SLEDAI) (19.2 ±8.7). Seven had DAH at initial diagnosis of lupus, 4 had recurrent episodes of DAH. Ten (52.6%) patients died. Clinical manifestations included fever (84.2%), cough (78.9%), dyspnea (100%), hemoptysis (73.7%). Organ involvement include nephritis in (14/19) 73.7%, CNS involvement (6/19) 31.6%, arthritis (9/19) 47.4%, mucocutaneous (13/19) 68.4%, myocarditis (5/19) 26.3%, pulmonary arterial hypertension (7/19) 36.8%, serositis (8/19) 42.1%, renal failure (8/19) 42.1%, autoimmune hemolytic anemia in (2/19) 10.5% patients. Leucopenia (10/19) 52.6%, thrombocytopenia (11/19) 57.9%, low complements in 14/15 patients, dsDNA positive in (16/19) 84.2%, aCL antibody positivity in 4/14, raised serum procalcitonin in 3/14. Mean erythrocyte sedimentation rate (ESR) was 52.9±41.6 mm first hour, mean creatinine 1.6±1.5 mg/dl. Concomitant infection was seen in 7 (36.8%) patients. Respiratory tract infection was seen in 5 (4 had bacterial, 1 had strongyloides) and 2 had systemic infection. Eleven (57.9%) required mechanical ventilation. All patients received intravenous methylprednisolone pulses, cyclophosphamide in (16/19) 84.2%, IVIg in 2, Rituximab in 1, plasmapheresis in 1 patient. Median follow up of survivors is 33 months, 2 lost to follow up.
Univariate analysis showed age and high ESR as independent risk factors associated with DAH. Mortality was associated with requirement of mechanical ventilation.
Conclusions DAH in SLE is rare, but associated with high mortality. It occurs in patients with high disease activity. Requirement of mechanical ventilation is associated with higher mortality.
Martínez-Martínez MU, Abud-Mendoza C. Predictors of mortality in diffuse alveolar haemorrhage associated with systemic lupus erythematosus. Lupus.2011;20:568–74.
Zamora MR, Warner ML, Tuder R, Schwarz MI. Diffuse alveolar hemorrhage and systemic lupus erythematosus: clinical presentation, histology, survival, and outcome. Medicine 1997; 76: 192–202.
Disclosure of Interest None declared