Autoimmune Pulmonary Alveolar Proteinosis Mimicking Connective Tissue Disease-Associated Interstitial Lung Disease: Case Presentation and Mini Literature Review

Tsai WH*, Hsieh SC and Li KJ

Department of Internal Medicine, Division of Rheumatology, Immunology, and Allergy, National Taiwan University Hospital, Taiwan

^*Correspondance to: Wan-Hao Tsai 

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Abstract:

Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by alveolar surfactant accumulation and macrophage dysfunction. Differentiating PAP from Connective Tissue Diseaserelated Interstitial Lung Disease (CTD-ILD) is crucial due to distinct treatment approaches and outcomes. ILD commonly complicates CTDs, but the specific ILD subtypes and their prevalence vary across different conditions. We present the case of a Taiwanese female initially diagnosed with CTD-ILD, later confirmed to have PAP through extensive evaluations. This case underscores the diagnostic challenge of distinguishing PAP from CTD-ILD, emphasizing the critical need to consider PAP in the differential diagnosis, given overlapping features. Timely and precise identification is essential for optimal management and outcomes, as the patient's remarkable improvement following whole lung lavage and Rituximab infusion demonstrates.

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Cite the Article:

Tsai WH, Hsieh SC, Li KJ. Autoimmune Pulmonary Alveolar Proteinosis Mimicking Connective Tissue Disease- Associated Interstitial Lung Disease: Case Presentation and Mini Literature Review. Ann Clin Case Rep. 2024; 9: 2558..