A Case of Hypertrophic Pachymeningitis Associated with Immunoglobulin-G4 and c-ANCA

Abstract

Hypertrophic pachymeningitis (HP) is characterized by inflammation of the dura mater. It has been described in the setting of numerous systemic inflammatory diseases including immunoglobulin G4 (IgG4)-related disease as well as granulomatosis with polyangiitis (GPA). In this case report, we describe a 48-year-old man presenting with headache who was found to have HP and had systemic features of both GPA and IgG4-related disease as well as seropositivity for both cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and IgG4. He was treated with prednisone and rituximab with improvement in his symptoms. Co-occurrence of IgG4 and ANCA against myeloperoxidase has been reported in other cases of HP. The overlap between IgG4 and ANCA has also been described in other systemic manifestations of the diseases. These reports suggest a clinical overlap between ANCA and IgG4-related disease, and the case presented herein suggests an overlap between GPA and IgG4-related disease.

The great imposter: A case report of IgG4‐RD hypertrophic pachymeningitis with skull lytic lesion and pulmonary nodules

“…He had systemic features of both GPA and IgG4‐related disease and seropositivity for both cytoplasmic antineutrophil cytoplasmic antibodies (c‐ANCAs) and IgG4. 20 He was treated with prednisone and rituximab with improvement in his symptoms.…”

“…In one report, a 48‐year‐old man presenting with a headache was found to have HP along with incidental bilateral pulmonary nodules. He had systemic features of both GPA and IgG4‐related disease and seropositivity for both cytoplasmic antineutrophil cytoplasmic antibodies (c‐ANCAs) and IgG4 20 . He was treated with prednisone and rituximab with improvement in his symptoms.…”

Hypertrophic Pachymeningitis with Characteristics of Both IgG4-related Disorders and Granulomatosis with Polyangiitis

“…Several cases of IgG4-related HP show serum positivity for ANCA ( 7 , 8 ). ANCA-positive IgG4-related HP showed fibroinflammatory lesions and numerous IgG4-positive plasma cells on a pathological examination of the dura mater ( 7 ); however, there have been no reports of MPO-ANCA-positive IgG4-related HP presenting as granulomatous inflammation in the dura mater.…”

“…Elevated serum IgG4 and infiltration of IgG4-positive plasma cells are characteristic features of IgG4-related diseases; however, GPA, EGPA, pulmonary sarcoidosis, and lymphoma have also been reported to present with similar pathological or serological features of IgG4-related diseases ( 6 ). Several patients with IgG4-related HP have demonstrated serum positivity for ANCA, and a few patients with HP have shown both clinical features of GPA and IgG4-related disease ( 7 , 8 ). Furthermore, the presence of granulomatous inflammation, including granuloma formation with multinucleated giant cells, has been reported in patients with IgG4-related HP whose ANCA status was uncertain ( 9 ).…”

The Great Imposter: A case Report of IgG4 RD -Hypertrophic Pachymeningitis with Skull Lytic lesion and Pulmonary Nodules

“…In this case, however, there is the commitment involvement of Meningeal disease with pulmonary nodules which has been uncommon. In one report, a 48-year-old man presenting with headache was found to have HP along with incidental bilateral pulmonary nodules and had systemic features of both GPA and IgG4-related disease as well as seropositivity for both cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and IgG4 (20). He was treated with prednisone and rituximab with improvement in his symptoms.…”