Background/Purpose: To evaluate the role of cytomegaloviral or Pneumocystic jiroveci pneumonia (CMV/PJP) in 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 patients had pathogens from BALF samples, and eight patients had CMV/PJP (Table 1). Although CMV/PJP was treated, the 90- and 180-day mortality rates of SLE patients with CMV/PJP were higher than those without these infections (90-day: 62.5% vs. 10.5%, p = 0.011; 180-day: 75% vs. 10.5%, p= 0.002). 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) 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). 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) (Table 2). Three of the seven patients receiving aggressive immunosuppression died with the presence of CMV/PJP.

Conclusion: Due to the high prevalence of CMV/PJP and its association with mortality, routine BALF analysis is recommended in all suitable SLE patients with PH. Use of aggressive immunosuppression does not benefit SLE patients with opportunistic infections during PH attack.

Table 1. Baseline characteristic of PH of SLE patients.

Values are presented as median (interquartile range) or number (percentage).

^*Within 3 months or ^**6 months before PH.

^†Only for patients ever supported with MV.

^‡p < 0.05 vs. patients without CMV pneumonia or PJP.

Table 2. Risk factor analysis for 90-day and 180-day mortality rates in SLE patients with PH

^*The only factor that remained significant when selecting variables with p < 0.05 in multivariable analysis. (90-day, OR: 14.2, 95% Cl: 1.83, 109.9; p = 0.011; 180-day, OR: 25.5, 95% Cl: 2.91, 223.3, p = 0.003)

ANCA, antineutrophil cytoplasmic antibody; APACHE II, Acute Physiology and Chronic Health Evaluation II; BAL, bronchoalveolar lavage; ECMO, extracorporeal membrane oxygenation; IgG, immunoglobulin G; IVIg, intravenous immunoglobulin; IS, immunosuppression; MV, Mechanical ventilation; MP, methylprednisolone; PDN, prednisolone; PH, pulmonary hemorrhage; SLEDAI-2K, Systemic Lupus Erythematosus Disease Activity Index 2000; SOFA, sequential organ failure assessment; WBC, white blood cell.