No abstract

IgG4-related disease (IgG4-RD) is a multi-organ, immune-mediated inflammatory condition of unknown etiology characterized by infiltration of tissues by IgG4 producing plasma cells. IgG4-related disease (IgG4-RD) can ideally affect any organs, but the involvement of the central nervous system (CNS) is a rare entity. We present a case of a 67-year-old male who presented with diplopia with imaging showing hypertrophic pachymeningitis (HPM) and was diagnosed with IgG4-RD of the CNS based on histopathology report.

Humoral immune responses can produce autoantibodies against self-cellular proteins and nucleic acids without the presence of autoimmune diseases. Numerous kinds of autoantibodies are detected in liver diseases such as viral hepatitis, alcoholic liver disease (ALD), and nonalcoholic fatty liver disease (NAFLD), where their production could be secondary to hepatocellular inflammation and necrosis. Hence, the presence of an autoantibody does not necessarily indicate the presence of autoimmune disease; nor does it predict its severity and potential response to therapy. In literature, the spectrum and methods of diagnosis of liver disease in lupus are well described. However, chronic liver disease can manifest with signs similar to those in lupus, and it is important to recognize that autoantibodies in patients with chronic liver disease can be seen without the presence of autoimmune rheumatic disease. In this report, we discuss a very interesting case of a middle-aged female with a history of ALD presenting with calciphylaxis, thrombocytopenia, hypocomplementemia, and positive serologies, but without any clinical evidence of autoimmune rheumatic disease.