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Case Reports
. 2024 Nov 28;16(11):e74669.
doi: 10.7759/cureus.74669. eCollection 2024 Nov.

Pulmonary Alveolar Proteinosis During Intensive Immunosuppressive Treatment for Acute Exacerbation of Interstitial Pneumonia: A Case Report and Literature Review

Atsushi Yanagisawa  1   2 , Hachiro Konaka  1 , Masaki Tanaka  1 , Shoichi Ihara  1 , Isao Tachibana  1
Affiliations
Case Reports

Pulmonary Alveolar Proteinosis During Intensive Immunosuppressive Treatment for Acute Exacerbation of Interstitial Pneumonia: A Case Report and Literature Review

Atsushi Yanagisawa et al. Cureus. .

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of surfactants in the alveoli. It has been suggested that immunosuppressants contribute to the development and exacerbation of PAP. Here, we report the case of a 73-year-old man who developed secondary PAP after intensive immunosuppressive treatment for acute exacerbation of interstitial pneumonia (IP). In this case, despite the improvement of the inflammatory response after immunosuppressive treatment, Krebs von den Lungen-6 (KL-6) continued to increase, leading to the diagnosis of PAP. De-escalation of immunosuppressive treatment improved the PAP, allowing him to be discharged from the hospital. Although KL-6 is a useful marker of IP, when IP appears to be refractory and KL-6 increases despite the improvement of other inflammatory markers, physicians should consider the development of PAP and perform proactive bronchoscopic evaluation.

Keywords: acute exacerbation of interstitial pneumonia; immunosuppressive treatment; krebs von den lungen-6; pulmonary alveolar proteinosis; secondary pulmonary alveolar proteinosis.

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Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Clinical course.
LDH: lactate dehydrogenase; CRP: C-reactive protein; KL-6: Krebs von den Lungen-6; CT: computed tomography; IP: interstitial pneumonia. All days are counted from the day of admission due to acute exacerbation of IP.
Figure 2
Figure 2. Cytopathological examination of the BAL on day 73.
BAL on day 73 (third bronchoscopy) showed abundant extracellular granular material (A, Papanicolaou stain) that was PAS positive. PAS-positive material is also present in alveolar macrophages (B, PAS stain). PAS: periodic acid-Schiff; BAL: bronchoalveolar lavage.
See this image and copyright information in PMC

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