Lymphoid interstitial pneumonia
General Information (adopted from Orphanet):
Synonyms, Signs: |
LIP Lymphocytic interstitial pneumonia |
Number of Symptoms | 45 |
OrphanetNr: | 79128 |
OMIM Id: |
247610
|
ICD-10: |
J84.1 |
UMLs: |
C0264511 |
MeSH: |
|
MedDRA: |
10062997 |
Snomed: |
44274007 |
Prevalence, inheritance and age of onset:
Prevalence: | No data available. |
Inheritance: |
|
Age of onset: |
|
Disease classification (adopted from Orphanet):
Parent Diseases: |
Idiopathic interstitial pneumonia
-Rare respiratory disease |
Comment:
The disease usually shows good response to combinative therapy of corticosteroids and cytotoxic agents. (PMID:19040067) |
Symptom Information:
|
(HPO:0012378) | Fatigue | 7360539 | IBIS | 50 / 7739 | ||
|
(HPO:0100759) | Clubbing of fingers | 7360539 | IBIS | 40 / 7739 | ||
|
(HPO:0100749) | Chest pain | 3453998 | IBIS | 92 / 7739 | ||
|
(HPO:0001217) | Clubbing | 1907689 | IBIS | 39 / 7739 | ||
|
(HPO:0002240) | Hepatomegaly | 7360539 | IBIS | 467 / 7739 | ||
|
(HPO:0002613) | Biliary cirrhosis | 3453998 | IBIS | 11 / 7739 | ||
|
(HPO:0012115) | Hepatitis | 3453998 | IBIS | 24 / 7739 | ||
|
(HPO:0001508) | Failure to thrive | 9361104 | IBIS | 454 / 7739 | ||
|
(HPO:0000961) | Cyanosis | 7360539 | IBIS | 60 / 7739 | ||
|
(HPO:0001640) | Cardiomegaly | 7360539 | IBIS | 81 / 7739 | ||
|
(HPO:0001708) | Right ventricular failure | 7360539 | IBIS | 11 / 7739 | ||
|
(HPO:0001650) | Aortic valve stenosis | 7360539 | IBIS | 49 / 7739 | ||
|
(HPO:0001649) | Tachycardia | 7360539 | IBIS | 53 / 7739 | ||
|
(HPO:0001648) | Cor pulmonale | 7360539 | IBIS | 16 / 7739 | ||
|
(HPO:0010702) | Increased antibody level in blood | Frequent [IBIS] | 19040067 | IBIS | 29 / 7739 | |
|
(HPO:0004313) | Decreased antibody level in blood | Occasional [IBIS] | 3453998 | IBIS | 47 / 7739 | |
|
(HPO:0001945) | Fever | 7360539 | IBIS | 218 / 7739 | ||
|
(HPO:0012398) | Peripheral edema | 7360539 | IBIS | 12 / 7739 | ||
|
(HPO:0002795) | Functional respiratory abnormality | 8069020 | IBIS | 13 / 7739 | ||
|
(HPO:0005952) | Decreased pulmonary function | 8069020 | IBIS | 8 / 7739 | ||
|
(HPO:0002206) | Pulmonary fibrosis | 8069020 | IBIS | 51 / 7739 | ||
|
(HPO:0002111) | Restrictive deficit on pulmonary function testing | 16571614 | IBIS | 25 / 7739 | ||
|
(HPO:0002094) | Dyspnea | Frequent [IBIS] | 19040067 | IBIS | 132 / 7739 | |
|
(HPO:0002207) | Diffuse reticular or finely nodular infiltrations | 19040067 | IBIS | 11 / 7739 | ||
|
(HPO:0012418) | Hypoxemia | 8069020 | IBIS | 18 / 7739 | ||
|
(HPO:0002093) | Respiratory insufficiency | 16571614 | IBIS | 410 / 7739 | ||
|
(HPO:0002875) | Exertional dyspnea | 8069020 | IBIS | 29 / 7739 | ||
|
(HPO:0006515) | Interstitial pneumonitis | 19040067 | IBIS | 13 / 7739 | ||
|
(HPO:0002090) | Pneumonia | 19040067 | IBIS | 59 / 7739 | ||
|
(HPO:0006527) | Lymphoid interstitial pneumonia | Very frequent [IBIS] | 19040067 | IBIS | 1 / 7739 | |
|
(HPO:0002789) | Tachypnea | 9361104 | IBIS | 48 / 7739 | ||
|
(HPO:0002113) | Pulmonary infiltrates | 19040067 | IBIS | 36 / 7739 | ||
|
(HPO:0006530) | Interstitial pulmonary disease | Very frequent [IBIS] | 24424167 | IBIS | 26 / 7739 | |
|
(HPO:0002103) | Abnormality of the pleura | 8069020 | IBIS | 58 / 7739 | ||
|
(HPO:0002729) | Follicular hyperplasia | 8069020 | IBIS | 5 / 7739 | ||
|
(HPO:0002716) | Lymphadenopathy | 8069020 | IBIS | 129 / 7739 | ||
|
(HPO:0002715) | Abnormality of the immune system | 9361104 | IBIS | 46 / 7739 | ||
|
(OMIM) | Cough, nonproductive | Frequent [IBIS] | 19040067 | IBIS | 5 / 7739 | |
|
(HPO:0012735) | Cough | Frequent [IBIS] | 19040067 | IBIS | 24 / 7739 | |
|
(OMIM) | Radiograph shows ground glass appearance | 15459629 | IBIS | 7 / 7739 | ||
|
(HPO:0002878) | Respiratory failure | 3453998 | IBIS | 57 / 7739 | ||
|
(MedDRA:10037833) | Rales | 8069020 | IBIS | 4 / 7739 | ||
|
(MedDRA:10067182) | Bronchial wall thickening | 15459629 | IBIS | 5 / 7739 | ||
|
(OMIM) | Honeycomb fibrosis, varying in age and location | 8069020 | IBIS | 6 / 7739 | ||
|
(HPO:0040088) | Abnormal lymphocyte count | 8069020 | IBIS | 3 / 7739 |
Associated genes:
ClinVar (via SNiPA)
Gene symbol | Variation | Clinical significance | Reference |
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