Pneumocystis jirovecii pneumonia plays an increasing role in patients with autoimmune disorders, due to more intensive immunosuppressive therapy. Humans seem to be the most important pathogen reservoir. Diseases are probably caused by airborne new infections. Cough, subfebrile temperature and dyspnea on exertion are the leading symptoms. In addition to imaging, in particular high-resolution computed tomography, pathogen detection by staining methods or molecular genetic methods plays the decisive role. Trimethoprim and sulfamethoxazole (TMP-SMX) is the most important medication for treatment. Adjuvant corticosteroid treatment is sometimes recommended, but evidence for benefits in patients with rheumatological disorders is not well documented. For patients on high-dose systemic corticosteroid treatment or intensive combined immunosuppression, primary prophylaxis is recommended by many experts. TMP-SMX remains the first-choice preventive treatment in these patients.
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