Cryptogenic organizing pneumonia
Synonyms: BOOP · Bronchiolitis obliterans organizing pneumonia · COP
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare form of idiopathic interstitial pneumonia characterized pathologically by organizing pneumonia (OP) that presents with nonspecific flu-like symptoms, as well as cough and dyspnea, and where no etiological agent is found.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Cryptogenic organizing pneumonia | BOOP · Bronchiolitis obliterans organizing pneumonia · COP |
| fr | Pneumopathie organisée cryptogénique | BOOP · Bronchiolite oblitérante avec organisation pneumonique · POC |
| de | Pneumonie, kryptogene organisierende | BOOP · Bronchiolitis obliterans mit organisierender Pneumonie · COP |
| es | Neumonía con organización criptogénica | BONO · Bronquiolitis obliterante con neumonía organizada · NOC |
| it | Polmonite criptogenica organizzata | Bronchiolite obliterante - polmonite organizzata |
| nl | Cryptogene organiserende pneumonie | BOOP · Bronchiolitis obliterans organiserende pneumonie · COP |
| pt | Pneumopatia criptogénica organizada | BOOP · Bronquiolite obliterante-pneumonia organizada · COP |
| pl | Kryptogenne organizujące się zapalenie płuc | Organizujące się zapalenie płuc z zarostowym zapaleniem oskrzelików |
| cs | Kryptogenní organizující se pneumonie | Bronchiolitis obliterans s organizující se pneumonií |
| uk | Криптогенна організуюча пневмонія | BOOP · Облітеруючий бронхіоліт, що організовує пневмонію · COP |
| zh | 隐源性机化性肺炎 | 闭塞性细支气管炎机化性肺炎 |
| tr | Kriptojenik organize pnömoni | COP · BOOP · Bronşiyolitis obliterans organize pnömoni (boop) |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J84.1 | NTBT | attributed | validated |
| ICD-11 | CB03.2 | E | specific | validated |
| MONDO | 0015264 | E | validated | |
| MeSH | D018549 | E | validated | |
| UMLS | C0242770 | E | validated | |
| GARD | 1620 | E | validated | |
| MedDRA | 10011496 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:98300Idiopathic interstitial pneumonia
rare respiratory diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
4 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
| Point prevalence | 1-9 / 1 000 000 | 0.81 / 100 000 | Spain | Value and class | Validated | 15127977[PMID] |
| Point prevalence | 1-9 / 1 000 000 | 0.92 / 100 000 | Greece | Value and class | Validated | 19345567[PMID] |
| Annual incidence | 1-9 / 100 000 | 1.1 / 100 000 | Iceland | Value and class | Validated | 18204109[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
27 annotations · validated 2019-11-06| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0012735 | Cough | |
| HP:0032177 | Parenchymal consolidation | |
| Frequent (79-30%) | ||
| HP:0002039 | Anorexia | |
| HP:0030830 | Crackles | |
| HP:0002094 | Dyspnea | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0025179 | Ground-glass opacification on pulmonary HRCT | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0011897 | Neutrophilia | |
| HP:0031246 | Nonproductive cough | |
| HP:0002091 | Restrictive ventilatory defect | |
| Occasional (29-5%) | ||
| HP:0032016 | Abnormal sputum | |
| HP:0031994 | Bronchial breath sound | |
| HP:0100749 | Chest pain | |
| HP:0000961 | Cyanosis | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0002105 | Hemoptysis | |
| HP:0001824 | Weight loss | |
| HP:0030828 | Wheezing | |
| Very rare (<4-1%) | ||
| HP:0002829 | Arthralgia | |
| HP:0012418 | Hypoxemia | |
| HP:0030166 | Night sweats | |
| HP:0025421 | Pneumomediastinum | |
| HP:0002107 | Pneumothorax | |
| HP:0002098 | Respiratory distress | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 29939651[PMID]_30322441[PMID]_31159568[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/1302
- This record as JSON: /data/d/1302.json