IgG4-related pachymeningitis
Findings
No curated finding names IgG4-related pachymeningitis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Idiopathic hypertrophic pachymeningitis (IHP) is a rare disorder causing inflammation and thickening of the outer layer (dura) of the brain and/or spinal cord. IHP can be widespread or cause tumor-like lesions. Before IHP can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections (syphilis, tuberculosis, and Lyme disease), and autoimmune diseases (rheumatoid arthritis, Sjogrenbs syndrome, Wegenerbs granulomatosis, and IgG4-related disease) must be ruled out. IHP often presents with headache and cranial nerve impairment. Treatment may involve prednisone and/or an immune suppressing drug. This treatment often improves symptoms, however complete recovery is rare. Surgery may be recommended for people with advanced or severe IHP. Some people with IHP have no symptoms and may not need treatment.
Definition from the Mondo Disease Ontology (MONDO:0018673), read 2026-09-29. CC BY 4.0.
Features
39 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal cerebrospinal fluid morphologyHPOHP:0002921
- Very frequent (80% to 99% of cases)
- Abnormal dura mater morphologyHPOHP:0010652
- Very frequent (80% to 99% of cases)
- Elevated circulating C-reactive protein concentrationHPOHP:0011227
- Frequent (30% to 79% of cases)
- HeadacheHPOHP:0002315
- Frequent (30% to 79% of cases)
- Increased circulating IgG4 concentrationHPOHP:0032300
- Frequent (30% to 79% of cases)
- Increased CSF protein concentrationHPOHP:0002922
- Frequent (30% to 79% of cases)
Show the remaining 27
- Abnormal temporal bone morphologyHPOHP:0009911
- Occasional (5% to 29% of cases)
- Abnormality of cervical plexusHPOHP:3000035
- Occasional (5% to 29% of cases)
- Abnormality of the brachial nerve plexusHPOHP:0045052
- Occasional (5% to 29% of cases)
- Abnormality of the cervical spineHPOHP:0003319
- Occasional (5% to 29% of cases)
- AphasiaHPOHP:0002381
- Occasional (5% to 29% of cases)
- ConfusionHPOHP:0001289
- Occasional (5% to 29% of cases)
Where it sits
Other names
1 name
Resolves to: IgG4-related pachymeningitis
- Also called
- idiopathic hypertrophic pachymeningitis