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lymphoid interstitial pneumonia - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

lymphoid interstitial pneumonia

MONDO:0009537

Also known as:diffuse hyperplasia of bronchus-associated lymphoid tissuelymphocytic interst. pneumonitislymphocytic interstitial pneumonia

Overview

Interstitial pneumonia characterized by the presence of bibasilar pulmonary interstitial infiltrates composed of lymphocytes and plasma cells. It may be associated with autoimmune and lymphoproliferative disorders. Signs and symptoms include fever, cough, and dyspnea. Symptomatic patients may require immunosuppressive treatment.

GeneticsAutosomal recessive

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include: Aortic valve stenosis, Lymphocytic interstitial pneumonia, Clubbing, and Cor pulmonale.

Organ System	Phenotype Count	Example Features
Heart and blood vessels	1	Aortic valve stenosis
Lungs and breathing

Diagnosis

Biomarker and diagnostic research for lymphoid interstitial pneumonia has been reported in the published literature.

Prognosis

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for lymphoid interstitial pneumonia.

37 publications have been identified in PubMed for lymphoid interstitial pneumonia. Research spans Case Report / Case Series (41%), Review / Meta-Analysis (32%), and Epidemiology / Natural History (14%).

Research Type	Count	% of Total
Patient case studies	15	41%

Data assembled from 5 of 12 sources · Last updated Sep 18, 2026, 7:13 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:247610

Orphanet

European rare disease database

Orphanet:79128

GARD

Genetic and Rare Diseases Info Center

GARD:16700

Questions for Your Care Team

→Should my partner and I meet with a genetic counselor to discuss carrier status and family planning?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Kisho

lymphoid interstitial pneumonia

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

lymphoid interstitial pneumonia

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications