immunoglobulin G4-related sclerosing disease IgG4-related pachymeningitis https://github.com/monarch-initiative/mondo/issues/6748 https://www.malacards.org/card/igg4_related_pachymeningitis idiopathic hypertrophic craniospinal pachymeningitis 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. MONDO:0018673 icd11.foundation:1140264879 idiopathic hypertrophic spinal pachymeningitis GARD:0013256 Orphanet:449427 UMLS:C4545992 idiopathic hypertrophic cranial pachymeningitis idiopathic hypertrophic pachymeningitis SCTID:762282007 MEDGEN:1627405 inflammatory disease