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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.
Estimated prevalence: Unknown (Unknown prevalence).
No clinical trials have been registered for IgG4-related pachymeningitis.
23 publications have been identified in PubMed for IgG4-related pachymeningitis. Research spans Case Report / Case Series (52%), Review / Meta-Analysis (22%), and Other (13%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 12 | 52% |
Data assembled from 3 of 12 sources · Last updated Sep 19, 2026, 2:58 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about IgG4-related pachymeningitis
5 |
22% |
Other research | 3 | 13% |
Disease patterns and progression | 2 | 9% |
Laboratory research | 1 | 4% |
Balasubramanian K (2026). [PMID: 41483370](https://pubmed.ncbi.nlm.nih.gov/41483370/). *Immunol Res*. [Review / Meta-Analysis]
Brescovit DO (2026). [PMID: 41455465](https://pubmed.ncbi.nlm.nih.gov/41455465/). *Clin Neurol Neurosurg*. [Epidemiology / Natural History]
Takahashi S (2026). [PMID: 41780981](https://pubmed.ncbi.nlm.nih.gov/41780981/). *Intern Med*. [Epidemiology / Natural History]
Balasubramanian K (2025). [PMID: 40288528](https://pubmed.ncbi.nlm.nih.gov/40288528/). *World Neurosurg*. [Review / Meta-Analysis]
Lai Y (2025). [PMID: 39805060](https://pubmed.ncbi.nlm.nih.gov/39805060/). *Neurology*. [Case Report / Case Series]
Kitkamolwat S (2025). [PMID: 40299826](https://pubmed.ncbi.nlm.nih.gov/40299826/). *PLoS One*. [Basic Science / Preclinical]
Ansari S (2025). [PMID: 41147905](https://pubmed.ncbi.nlm.nih.gov/41147905/). *Radiology*. [Case Report / Case Series]
Suda T (2025). [PMID: 38839333](https://pubmed.ncbi.nlm.nih.gov/38839333/). *Intern Med*. [Case Report / Case Series]
Pobudejski P (2025). [PMID: 41562813](https://pubmed.ncbi.nlm.nih.gov/41562813/). *Reports (MDPI)*. [Case Report / Case Series]
Terrim S (2025). [PMID: 39556369](https://pubmed.ncbi.nlm.nih.gov/39556369/). *JAMA Neurol*. [Review / Meta-Analysis]
AI-curated news mentioning IgG4-related pachymeningitis
Updated Aug 8, 2026
A systematic review has been published on IgG4-related spinal hypertrophic pachymeningitis, providing insights into its clinical features and management. This research contributes to the understanding of this rare condition and may guide future studies.