Compressive myelopathy: A rare clinical presentation of IgG4-related disease

IgG4-related disease (IgG4RD) is a new entry into the field of rheumatology characterized by tumefactive lesions, storiform fibrosis, and involvement of more than one organ. The most common organ involved is the pancreas which often coexist with mass lesions in other organs. We report a case where IgG4RD presented with the involvement of aorta and meninges in the form of periaortitis and hypertrophic pachymeningitis respectively, a rare coexistence in IgG4RD. The patient responded well to rituximab in the initial visit, however during subsequent recurrence, the response was not significant. We also discuss the immunosuppressants used in IgG4 RD and the variability in clinical response during various stages of disease.