Desquamative interstitial pneumonia

Orpha code: 98852OMIM code: 263000

Definition

A rare idiopathic interstitial pneumonia characterized by extensive, diffuse intra-alveolar accumulation of pigment-laden macrophages, most commonly associated with long-term exposure to tobacco smoke. Patients present with slowly progressive shortness of breath on exertion and chronic cough with bilateral crackles. Digital clubbing is also frequently observed. Pulmonary function test reveals a restrictive pattern. Computed tomography typically shows diffuse ground-glass opacities with subpleural and lower zone predominance.

Disease data
Classification

Disease

ORPHA code
98852
OMIM code
263000
ICD10 code
J84.1
ICD11 code
-

No additional description.

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