ORPHA:79128

Lymphoid interstitial pneumonia

Synonyms: Lymphocytic interstitial pneumonia

Type: Disease
Group: Disorder
Inheritance: Not applicable
Age of onset: All ages
Status: active

A rare idiopathic interstitial pneumonia characterized by a diffuse, dense, polyclonal lymphoid cell infiltration of the pulmonary interstitium and air spaces, with high prevalence in patients with immune dysregulation. Presenting symptoms are non-specific and include dyspnea and cough. The clinical course is highly variable, ranging from spontaneous resolution to progressive, fatal respiratory failure.

Names in Orphadata languages

12 of 12 languages

Lang	Name	Synonyms
en	Lymphoid interstitial pneumonia	Lymphocytic interstitial pneumonia
fr	Pneumopathie interstitielle lymphocytaire
de	Pneumonie, interstitielle, lymphoide
es	Neumonía linfoide intersticial
it	Polmonite interstiziale linfoide
nl	Lymfoïde interstitiële pneumonie
pt	Pneumonia intersticial linfóide
pl	Limfatyczne śródmiąższowe zapalenie płuc	Limfocytarne śródmiąższowe zapalenie płuc
cs	Lymfoidní intersticiální pneumonie	Lymfatická intersticiální pneumonie
uk	Лімфоїдна інтерстиціальна пневмонія	Лімфоцитарна інтерстиціальна пневмонія
zh	淋巴样间质性肺炎	淋巴细胞性间质性肺炎
tr	Lenfoid interstisyel pnömoni	Lenfositik interstisyel pnömoni

Definition in:

Cross-references

qualifier = Orphanet mapping relation

Source	Code	Qualifier	ICD relation	Validation
OMIM	247610	E		validated
ICD-10	J84.1	NTBT	attributed	validated
ICD-11	CB03.5	E	specific	validated
MONDO	0009537	E		validated
MeSH	C562489	E		validated
UMLS	C0264511	E		validated
MedDRA	10062997	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):

ORPHA:97955Rare respiratory disease

Parents in the classifications (1):

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

No children.

Genes

0 direct

Orphanet records no gene directly on this code.

Prevalence and incidence

1 record

Type	Class	Value	Area	Qualification	Validation	Source
Point prevalence	Unknown	—	Worldwide	Class only	Not yet 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.

HPO phenotypes

34 annotations · validated 2021-09-29

HPO	Term (as annotated by Orphanet)	Diagnostic
Very frequent (99-80%)
HP:0012735	Cough
Frequent (79-30%)
HP:0030057	Autoimmune antibody positivity
HP:0002960	Autoimmunity
HP:0001217	Clubbing
HP:0030830	Crackles
HP:0045051	Decreased DLCO
HP:0002094	Dyspnea
HP:0000964	Eczematoid dermatitis
HP:0001508	Failure to thrive
HP:0012378	Fatigue
HP:0025179	Ground-glass opacification on pulmonary HRCT
HP:0012418	Hypoxemia
HP:0005948	Multiple pulmonary cysts
HP:0032177	Parenchymal consolidation
HP:0002091	Restrictive ventilatory defect
HP:0031691	Severe viral infection
HP:0030828	Wheezing
Occasional (29-5%)
HP:0003549	Abnormality of connective tissue
HP:0002110	Bronchiectasis
HP:0025180	Centrilobular ground-glass opacification on pulmonary HRCT
HP:0000105	Enlarged kidney
HP:0001945	Fever
HP:0002240	Hepatomegaly
HP:0002721	Immunodeficiency
HP:0001097	Keratoconjunctivitis sicca
HP:0100721	Mediastinal lymphadenopathy
HP:0030950	Pulmonary venous hypertension
HP:0030880	Raynaud phenomenon
HP:0011947	Respiratory tract infection
HP:0001370	Rheumatoid arthritis
HP:0000988	Skin rash
HP:0025178	Subpleural interstitial thickening
HP:0001824	Weight loss
Very rare (<4-1%)
HP:0002206	Pulmonary fibrosis

Run the differential on the 1 obligate / very frequent terms

Annotation source: 31615921[PMID]_32040879[PMID]_33364263[PMID]

Sources for this card

Orphanet page: orpha.net/en/disease/detail/79128
This record as JSON: /data/d/79128.json