ORPHA:79126

Acute interstitial pneumonia

Synonyms: Acute interstitial pneumonitis · Hamman-Rich syndrome

A rare, rapidly progressive, and histologically distinct, form of idiopathic interstitial pneumonia.

Names in Orphadata languages

12 of 12 languages

Lang	Name	Synonyms
en	Acute interstitial pneumonia	Acute interstitial pneumonitis · Hamman-Rich syndrome
fr	Pneumopathie interstitielle aiguë	Pneumonite interstitielle aiguë · Syndrome de Hamman-Rich
de	Pneumonie, interstitielle, akute	Hamman-Rich-Syndrom
es	Neumonía intersticial aguda	Neumonitis intersticial aguda · Síndrome de Hamman-Rich
it	Polmonite acuta interstiziale	Sindrome di Hamman-Rich
nl	Acute interstitiële pneumonie	Acute interstitiële pneumonitis · Syndroom van Hamman-Rich
pt	Pneumonia intersticial aguda	Pneumonite intersticial aguda · Síndrome de Hamman-Rich
pl	Ostre śródmiąższowe zapalenie płuc	Ostre śródmiąższowe zapalenie płuc · Zespół Hammana i Richa
cs	Akutní intersticiální pneumonie	Akutní intersticiální pneumonitida · Hammanův-Richův syndrom · Hamman-Richův syndrom
uk	Гостра інтерстиціальна пневмонія	Гострий інтерстиціальний пневмоніт · Синдром Хаммана-Річа
zh	急性间质性肺炎	急性间质性肺炎
tr	Akut interstisyel pnömoni	Hamman-Rich sendromu · Akut interstisyel pnömoni

Definition in:

qualifier = Orphanet mapping relation

Source	Code	Qualifier	ICD relation	Validation
OMIM	178500	NTBT		validated
ICD-10	J84.1	NTBT	inclusion	validated
ICD-11	CB03.0	E	specific	validated
MONDO	0019203	E		validated
UMLS	C0085786	E		validated
GARD	12835	E		validated
MedDRA	10066728	E		validated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

ORPHA:98300Idiopathic interstitial pneumonia
rare respiratory diseases; rare transplant-related disorders

No children.

0 direct

Orphanet records no gene directly on this code.

1 record

Type	Class	Value	Area	Qualification	Validation	Source
Point prevalence	1-9 / 100 000	3.8 / 100 000	Europe	Value and class	Validated	ORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

33 annotations · validated 2020-02-12

HPO	Term (as annotated by Orphanet)	Diagnostic
Very frequent (99-80%)
HP:0002110	Bronchiectasis
HP:0002094	Dyspnea
HP:0025179	Ground-glass opacification on pulmonary HRCT
HP:0012418	Hypoxemia
HP:0030879	Interlobular septal thickening on pulmonary HRCT
HP:0025392	Nodular pattern on pulmonary HRCT
HP:0032177	Parenchymal consolidation
HP:0025177	Peribronchovascular interstitial thickening
HP:0002113	Pulmonary infiltrates
HP:0002878	Respiratory failure
HP:0025393	Reticulonodular pattern on pulmonary HRCT
Frequent (79-30%)
HP:0030830	Crackles
HP:0000961	Cyanosis
HP:0045051	Decreased DLCO
HP:0012378	Fatigue
HP:0001945	Fever
HP:0000822	Hypertension
HP:0031246	Nonproductive cough
HP:0002202	Pleural effusion
HP:0002789	Tachypnea
Occasional (29-5%)
HP:0002829	Arthralgia
HP:0100750	Atelectasis
HP:0100749	Chest pain
HP:0011227	Elevated circulating C-reactive protein concentration
HP:0003259	Elevated circulating creatinine concentration
HP:0003565	Elevated erythrocyte sedimentation rate
HP:0002716	Lymphadenopathy
HP:0003326	Myalgia
HP:0001698	Pericardial effusion
HP:0012398	Peripheral edema
HP:0002206	Pulmonary fibrosis
HP:0031851	Reduced hematocrit
HP:0031631	Subpleural honeycombing

Annotation source: 31333914[PMID]_29159034[PMID]_19095855[PMID]