Background/Purpose:  Pneumocystis pneumonia (PCP) is one of the severe opportunistic infections in immunocompromised patients. PCP is still a leading cause of death in patients with connective tissue diseases treated with glucocorticoid, immunosuppressant and biologic agents. However, the mortality rate and factors related to death for PCP remain unclear. The aim of this study is to identify the prognostic factors for PCP in immunosuppressed patients with connective tissue diseases.

Methods:  A total of 52 patients with connective tissue diseases developing PCP were included in this study. PCP was diagnosed by clinical symptoms, radiological findings, respiratory specimens (the microscopic demonstration of Pneumocystis jirovecii or pneumocystis-specific nested polymerase chain reaction) and serum β-D glucan. The patients were divided into two groups according to the outcome (Survivors group or Non-survivors group). Baseline demographics, treatment for the underlying connective tissue disease, laboratory findings at the diagnosis of PCP, and treatment for PCP were compared between the two groups.

Results:   Of 52 patients with PCP, 6 deceased and a mortality rate was 11.5%. There were no significant differences in age, gender, type of connective tissue disease, previous immunosuppressive treatments between survivors and non-survivors. The treatment regimen for PCP was not different between the two groups. The initial trimethoprim-sulfamethoxazole treatment failure was also not statistically different between the two groups. In univariate analysis, while the levels of serum β-D glucan, KL-6, IgG and PaO2, and the number of lymphocytes were not statistically different, the lower level of serum albumin, and higher levels of serum C-reactive protein and lactate dehydrogenase were significantly associated with mortality. Multivariate analysis showed that the lower level of serum albumin was an independent poor prognostic factor for the death with PCP.

Conclusion:  The low serum albumin is an independent prognostic factor for mortality in PCP developed in immunosuppressed patients with connective tissue diseases.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/mortality-and-prognostic-factors-of-pneumocystis-pneumonia-in-patients-with-connective-tissue-diseases/