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Published on: March 17, 2014
Opportunistic infections in patients with pulmonary alveolar proteinosis
Ankit D Punatar1, Shimon Kusne, Janis E Blair
1Department of Internal Medicine, Mayo Clinic, Phoenix, AZ, USA.
Objectives:
To describe the demographics, clinical manifestations, treatment, and outcomes of patients with pulmonary alveolar proteinosis (PAP) who developed an opportunistic infection with Nocardia spp., mycobacteria or fungal pathogens.
Methods:
A case of PAP and Nocardia spp. brain abscess is described. A comprehensive review of the English-language literature was conducted to identify all reported cases of PAP and opportunistic infections between 1950 and July, 2010.
Results:
Seventy five cases were reviewed. Thirty two patients (43%) had nocardial infection, 28 (37%) mycobacterial infection, and 15 (20%) fungal infection. Thirty nine patients (65%) were male. Seventeen patients (23%) were immunosuppressed. Twenty patients (27%) were active smokers. PAP was the initial diagnosis in 19 patients (33%), while infection presented first in 23 patients (40%); 16 patients (27%) had a concurrent diagnosis of PAP and infection. The average interval between PAP diagnosis and an opportunistic infection was 16 months. Lungs were the most common site of infection; extra-pulmonary infection was present in 27 patients (32%). Thirty nine patients (57%) survived through the follow-up period, while 31 died.
Conclusions:
Opportunistic infections can either precede or follow a diagnosis of PAP. PAP should be considered in apparently immunocompetent patients who present with an opportunistic infection and diffuse alveolar infiltrates.
Insights
Pulmonary alveolar proteinosis (PAP) patients frequently develop opportunistic infections like nocardial, mycobacterial, or fungal pathogens, which can appear before or after PAP diagnosis. Early consideration of PAP is vital for patients with unexplained infections and lung infiltrates.
Area of Science:
- Pulmonary Medicine
- Infectious Diseases
- Critical Care
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare lung disorder.
- Opportunistic infections pose a significant threat to PAP patients.
Observation:
- A literature review identified 75 cases of PAP with opportunistic infections (Nocardia, mycobacteria, fungi).
- Infections occurred before, after, or concurrently with PAP diagnosis.
- Extra-pulmonary involvement was noted in 32% of cases.
Findings:
- Nocardial infections were most common (43%), followed by mycobacterial (37%) and fungal (20%).
- Over half of the patients (57%) survived, but 31 patients died.
- Immunosuppression and smoking were noted risk factors.
Implications:
- PAP should be considered in immunocompetent individuals presenting with opportunistic infections and diffuse alveolar infiltrates.
- The timing of opportunistic infections relative to PAP diagnosis is variable.
- Understanding these associations can improve diagnostic and treatment strategies for PAP patients.
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