Non-specific interstitial pneumonia

General Information (adopted from Orphanet):

Synonyms, Signs: NSIP
Non-specific idiopathic interstitial pneumonia
Number of Symptoms 43
OrphanetNr: 91364
OMIM Id:
ICD-10: J84.8
UMLs: C1290344
MeSH:
MedDRA:
Snomed: 129452008

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Sporadic
Age of onset:

Disease classification (adopted from Orphanet):

Parent Diseases: Idiopathic interstitial pneumonia
 -Rare respiratory disease

Comment:

Non-specific interstitial pneumonia (NSIP) belongs to the class of idiopathic interstitial pneumonia characterized histopathologically by temporally uniform lesions, in contrast to the temporal heterogeneity of usual interstitial pneumonia. The specific histological pattern is composed of varying degrees of alveolar wall inflammation or fibrosis (PMID:26100347, 18829672). Honeycombing and consolidation are relatively infrequent in patients with NSIP (PMID: 18829672, 12554898). Among patients with initial diagnosis of idiopathic NSIP, some develop clinical manifestation of autoimmune diseases such as collagen vascular disease or autoimmune thyroiditis during follow-up (PMID: 21273390, PMID:18829672, 11790668). There is neither sexual predominance nor association with cigarette smoking and prognosis is more favorable than for the other idiopathic interstitial pulmonary diseases (PMID:11790668).

Symptom Information: Sort by abundance 

1
 (HPO:0012378) Fatigue Occasional [IBIS] 12% (n=33) 26100347 IBIS 50 / 7739
2
 (HPO:0002829) Arthralgia 26100347 IBIS 79 / 7739
3
 (HPO:0100759) Clubbing of fingers 11790668 IBIS 40 / 7739
4
 (HPO:0100749) Chest pain Occasional [IBIS] 6% (n=33) 26100347 IBIS 92 / 7739
5
 (HPO:0001386) Joint swelling 26100347 IBIS 7 / 7739
6
 (HPO:0001824) Weight loss 11790668 IBIS 42 / 7739
7
 (HPO:0000988) Skin rash 26100347 IBIS 98 / 7739
8
 (HPO:0001640) Cardiomegaly 24001730 IBIS 81 / 7739
9
 (HPO:0011991) Abnormal neutrophil cell number Frequent [IBIS] 89% (n=19) 12952254 IBIS 3 / 7739
10
 (HPO:0001945) Fever Occasional [IBIS] 27% (n=33) 26100347 IBIS 218 / 7739
11
 (HPO:0100598) Pulmonary edema 26029588 IBIS 17 / 7739
12
 (HPO:0012415) Abnormal blood gas level 26100347 IBIS 9 / 7739
13
 (HPO:0002113) Pulmonary infiltrates 11790668 IBIS 36 / 7739
14
 (HPO:0002109) Abnormality of the bronchi 26029588 IBIS 6 / 7739
15
 (HPO:0002094) Dyspnea Very frequent [IBIS] 97% (n=33) 26100347 IBIS 132 / 7739
16
 (HPO:0002795) Functional respiratory abnormality 11790668 IBIS 13 / 7739
17
 (HPO:0002206) Pulmonary fibrosis Frequent [IBIS] 87% (n=83) 18829672 IBIS 51 / 7739
18
 (HPO:0002093) Respiratory insufficiency 18829672 IBIS 410 / 7739
19
 (HPO:0002090) Pneumonia 26100347 IBIS 59 / 7739
20
 (HPO:0002110) Bronchiectasis Frequent [IBIS] 76% (n=33) 26100347 IBIS 73 / 7739
21
 (HPO:0002111) Restrictive deficit on pulmonary function testing Frequent [IBIS] 83% (n=30) 26100347 IBIS 25 / 7739
22
 (HPO:0002207) Diffuse reticular or finely nodular infiltrations Frequent [IBIS] 73% (n=33) 26100347 IBIS 11 / 7739
23
 (HPO:0002792) Reduced vital capacity 18829672 IBIS 17 / 7739
24
 (HPO:0002103) Abnormality of the pleura Frequent [IBIS] 67% (n=33) 26100347 IBIS 58 / 7739
25
 (HPO:0012418) Hypoxemia 11790668 IBIS 18 / 7739
26
 (HPO:0006530) Interstitial pulmonary disease 24424167 IBIS 26 / 7739
27
 (HPO:0006515) Interstitial pneumonitis 26029588 IBIS 13 / 7739
28
 (HPO:0002923) Rheumatoid factor positive Occasional [IBIS] 21% (n=28) 26100347 IBIS 5 / 7739
29
 (HPO:0003493) Antinuclear antibody positivity Frequent [IBIS] 76% (n=25) 26100347 IBIS 15 / 7739
30
 (OMIM) Type II pneumocyte hyperplasia 11790668 IBIS 4 / 7739
31
 (MedDRA:10065906) Carbon monoxide diffusing capacity decreased Very frequent [IBIS] 11790668 IBIS 8 / 7739
32
 (OMIM) Sputum production Occasional [IBIS] 24% (n=33) 26100347 IBIS 3 / 7739
33
 (OMIM) Raynaud syndrome 26100347 IBIS 3 / 7739
34
 (HPO:0040088) Abnormal lymphocyte count Frequent [IBIS] 88% (n=25) 26100347 IBIS 3 / 7739
35
 (OMIM) Honeycomb fibrosis, varying in age and location Occasional [IBIS] 18829672, 11790668 IBIS 6 / 7739
36
 (OMIM) Decreased CD4+/CD8+ ratio Very frequent [IBIS] 96% (n=25) 24424167 IBIS 2 / 7739
37
 (OMIM) Reduced diffusion capacity Very frequent [IBIS] 100% (n=27) 26100347 IBIS 8 / 7739
38
 (MedDRA:10062049) Lymphocytic infiltration 26100347 IBIS 9 / 7739
39
 (HPO:0012735) Cough Very frequent [IBIS] 94% (n=33) 26100347 IBIS 24 / 7739
40
 (OMIM) Radiograph shows ground glass appearance Frequent [IBIS] 64% (n=33) 26100347 IBIS 7 / 7739
41
 (HPO:0030166) Night sweats 26029588 IBIS 3 / 7739
42
 (OMIM) Inspiratory crackles (50%) 11790668 IBIS 4 / 7739
43
 (MedDRA:10025080) Lung consolidation Frequent [IBIS] 36% (n=33) 26100347 IBIS 5 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: