IgG4-related aortitis
disease diseaseOn this page
Also known as IgG4-related periaortitis
Summary
IgG4-related aortitis (MONDO:0018672) is a disease. A subtype of immunoglobulin G4-related sclerosing disease — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: Unknown (Worldwide) [Orphanet-validated]
- Phenotypes (HPO): 25
Clinical features
Signs & symptoms
Clinical features (HPO)
25 HPO clinical features (Orphanet curated; top 25 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0010702 | Increased circulating antibody level | Very frequent (80-99%) |
| HP:0032300 | Increased circulating IgG4 level | Very frequent (80-99%) |
| HP:0001824 | Weight loss | Frequent (30-79%) |
| HP:0001945 | Fever | Frequent (30-79%) |
| HP:0002027 | Abdominal pain | Frequent (30-79%) |
| HP:0002099 | Asthma | Frequent (30-79%) |
| HP:0002960 | Autoimmunity | Frequent (30-79%) |
| HP:0003212 | Increased circulating IgE level | Frequent (30-79%) |
| HP:0003419 | Low back pain | Frequent (30-79%) |
| HP:0003493 | Antinuclear antibody positivity | Frequent (30-79%) |
| HP:0003565 | Elevated erythrocyte sedimentation rate | Frequent (30-79%) |
| HP:0011227 | Elevated circulating C-reactive protein concentration | Frequent (30-79%) |
| HP:0012393 | Allergy | Frequent (30-79%) |
| HP:0012649 | Increased inflammatory response | Frequent (30-79%) |
| HP:0012727 | Thoracic aortic aneurysm | Frequent (30-79%) |
| HP:0032061 | Hypereosinophilia | Frequent (30-79%) |
| HP:0000126 | Hydronephrosis | Occasional (5-29%) |
| HP:0002647 | Aortic dissection | Occasional (5-29%) |
| HP:0004431 | Complement deficiency | Occasional (5-29%) |
| HP:0004970 | Ascending tubular aorta aneurysm | Occasional (5-29%) |
| HP:0005214 | Intestinal obstruction | Occasional (5-29%) |
| HP:0012303 | Abnormal aortic arch morphology | Occasional (5-29%) |
| HP:0031252 | Dilated left subclavian artery | Occasional (5-29%) |
| HP:0032230 | Cytoplasmic antineutrophil antibody positivity | Occasional (5-29%) |
| HP:0430021 | Abnormal common carotid artery morphology | Occasional (5-29%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | IgG4-related aortitis |
| Mondo ID | MONDO:0018672 |
| Orphanet | 449400 |
| ICD-11 | 593151236 |
| UMLS | C5569008 |
| MedGen | 1800431 |
| GARD | 0021883 |
| Is cancer (heuristic) | no |
Also known as: IgG4-related periaortitis
Disease family
This is a subtype of immunoglobulin G4-related sclerosing disease. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › immune system disorder › autoimmune disease › immunoglobulin G4-related sclerosing disease › IgG4-related aortitis
Related subtypes (13): autoimmune pancreatitis, IgG4-related mesenteritis, IgG4-related sclerosing cholangitis, IgG4-related kidney disease, IgG4-related pachymeningitis, IgG4-related submandibular gland disease, IgG4-related ophthalmic disorder, eosinophilic angiocentric fibrosis, primary cutaneous plasmacytosis, cutaneous pseudolymphoma, IgG4-related retroperitoneal fibrosis, IgG4-related mediastinitis, IgG4-related thyroid disease
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 0.
Related Atlas pages
No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.