What question did this study set out to answer?

The study aims to understand the clinical course and identify prognostic factors related to severe Pneumocystis pneumonia in patients with autoimmune or inflammatory diseases.

May 31, 2026Open Access

Clinical course, treatment and outcome of severe pneumocystis pneumonia in patients with autoimmune or inflammatory diseases: a prospective study

Key Points

The study aims to understand the clinical course and identify prognostic factors related to severe Pneumocystis pneumonia in patients with autoimmune or inflammatory diseases.
Conducted a 5-year prospective single-center study in the ICU
Enrolled patients with severe Pneumocystis pneumonia and autoimmune diseases
Collected clinical, laboratory, radiological, therapeutic, and outcome data
Initial treatment failure occurred in 48.6%, linked to various risk factors including severe hypoxemia (OR 6.873, 95% CI 1.746–31.048, p = 0.006).
28-day ICU mortality was 57%, predicted by several variables including initial treatment failure (OR 10.591, 95% CI 1.926-45.768, p = 0.013).
Follow-up imaging showed architectural distortion or worsening fibrosis in over 50% of patients.

Abstract

Background The morbidity and mortality of Pneumocystis pneumonia (PCP) are rising among patients with autoimmune or inflammatory diseases (AID). Prospective studies focusing on severe PCP in this population remain scarce. A better understanding of the disease course and prognostic factors is urgently needed. Methods A 5-year prospective single-center study in the ICU enrolled AID patients with severe PCP. Clinical, laboratory, radiological, therapeutic, and outcome data were collected prospectively. Multivariable regression was used to identify risk factors for initial treatment failure and 28-day ICU mortality. Results Among 107 enrolled patients, the mean PaO 2 /FiO 2 ratio was 167 mmHg, and 76.6% of patients required mechanical ventilation at ICU admission. Pulmonary coinfection was detected in 46 (43.0%) patients. Initial treatment failure occurred in 48.6% and was associated with underlying connective tissue disease-interstitial lung disease (OR 3.241, 95% CI 1.337-25.147, p = 0.004), coinfection (OR, 5.419; 95% CI, 1.565-38.759; p = 0.008), and severe hypoxemia on ICU admission (OR 6.873, 95% CI 1.746–31.048, p = 0.006). Follow-up pulmonary imaging revealed architectural distortions or worsening fibrosis in over 50% of patients. The 28-day ICU mortality was 57.0%; independently predicted by PaO 2 /FiO 2 ratio 100 mmHg at PCP onset (OR 8.119, 95% CI 1.543-32.871, p = 0.007), initial treatment failure (OR 10.591, 95% CI 1.926-45.768, p = 0.013), persistent CD 4 + T-cell counts 100 cells/μL (OR 4.137, 95% CI 1.219- 62.152, p = 0.021), fibrosis score 260 during early follow-up (OR 5.354, 95% CI 1.513-19.756, p = 0.008), and new-onset shock during the ICU stay (OR 2.147, 95% CI 1.237-39.157, p = 0.018). Conclusions PCP is life-threatening in AID patients. Beyond baseline hypoxemia, early follow-up variables, particularly initial treatment failure and progressive pulmonary fibrosis, are critical prognostic indicators, warranting further research to drive understanding of their causes.

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