Non-specific interstitial pneumonia

Orpha code: 91364OMIM code:

Definition

A rare idiopathic interstitial pneumonia characterized by temporally uniform alveolar and interstitial mononuclear cell inflammation (cellular type) and/or fibrosis of the alveolar walls (fibrotic type) with preserved alveolar architecture. Other types of interstitial lung disease must be excluded. Symptoms are non-specific and include dyspnea, cough, and often constitutional symptoms such as fever and fatigue. Pulmonary function test reveals a restrictive pattern. Computed tomography shows predominantly lower lobe subpleural reticular changes, traction bronchiectasis, and ground-glass opacities. The cellular type of the disease is less common but carries a better prognosis.

Disease data
Classification

Disease

Synonyms
NSIP
Niespecyficzne idiopatyczne śródmiąższowe zapalenie płuc
NSIP
Non-specific idiopathic interstitial pneumonia
ORPHA code
91364
OMIM code
-
ICD10 code
J84.8
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl