Diffuse alveolar haemorrhage in systemic lupus erythematosus patients

Pulmonary haemorrhage (PH) in systemic lupus erythematosus (SLE) is a rare but potentially fatal complication due to its high mortality. Early treatment benefits the outcome. Reports on predictive factors of PH in SLE patients are scarce.

To describe a case series of PH in SLE patients that were attended in the Rheumatology Section of the J. M. Cullen Hospital and to compare this data with published results.

Patients with SLE (1982-1997 ACR criteria) and PH diagnosed by clinical criteria (cough, dyspnoea, haemoptysis), haemoglobin below 12 g/dL or drop greater than 2 points, new radiological infiltrate and bronchioalveolar lavage, monitored between June 1987 and December 2019 were studied. Demographic, clinical, laboratory, treatment and prognosis data related to PH were analysed.

From a database of 306 SLE patients, 25 (8.2%) developed 29 episodes of PH. PH was the first manifestation of SLE in 8 patients. Renal involvement was the most frequent manifestation prior to the development of PH. SLE activity (measured by SLEDAI) was high during the episodes (mean: 16.8). Renal failure (p = .027) and mechanical respiratory support (p = .006) were related to mortality (40.7%) with statistical significance. Patients with SLEDAI higher to 10 at SLE onset showed more likelihood of developing PH. The OR was 2.68 (p = .046).

Although treatment in SLE has progressed in recent years, PH continues to be a rare and severe complication of this disease. When a PH is suspected, studies to confirm it must be done rapidly, since early diagnosis and aggressive treatment have been shown to improve survival. We observed that patients with renal involvement and mechanical respiratory support had higher mortality than SLE patients without them.