Objective.To evaluate the role of cytomegaloviral or Pneumocystis jiroveci pneumonia (CMV/PJP) in systemic lupus erythematosus (SLE) patients with pulmonary hemorrhage (PH).Methods.We retrospectively examined hospital records for 27 SLE patients with PH who received bronchoalveolar lavage fluid (BALF) analyses. Clinical profile and mortality rates were compared between groups with and without CMV/PJP. Risk factors for PH-related mortality were analyzed.Results.Among 27 SLE patients with PH, 15 had pathogens from BALF samples, and 8 had CMV/PJP. Although CMV/PJP was treated, the RR for 90- and 180-day mortality rates of SLE patients with CMV/PJP were higher than those without these infections (5.94, 95% CI 1.44–24.48; 7.13, 95% CI 1.81–28.06, respectively). Risk factors for 90- and 180-day mortality were presence of CMV/PJP (OR 14.2, 95% CI 1.83–109.9; OR 25.5, 95% CI 2.91–223.3, respectively) and use of pulse methylprednisolone for PH treatment (OR 12.0, 95% CI 1.48–97.2; OR 8.5, 95% CI 1.13–63.9, respectively). Factors increasing the 90-day mortality rate were duration of mechanical ventilation exceeding 14 days (OR 11.1, 95% CI 1.11–112.0) and use of aggressive immunosuppression close to PH onset (OR 7.56, 95% CI 1.09–52.4). Three of the 7 patients receiving aggressive immunosuppression died with the presence of CMV/PJP.Conclusion.Owing to the high prevalence of CMV/PJP and its association with mortality, routine BALF analysis is recommended for all suitable SLE patients with PH. Use of aggressive immunosuppression does not benefit SLE patients with opportunistic infections during PH attack.
Effective treatment with double filtration plasmapheresis (DEPP) and high dose intravenous gammaglobulin therapy in a pregnant systemic lupus erythematosus(SLE) patient.