Introduction

Immunoglobulin G4-related disease (IgG4-RD) is a disorder that causes chronic inflammation and fibrosis by involving a variety of organs in the body. IgG4-RD commonly presents with mass-like swelling of an organ by dense infiltration of lymphocytes and IgG4-positive plasma cells arranged in a fibrosis storiform shape 1[Stone, 2012 #165]. Also, IgG4-RD is related to mildly infiltration of eosinophils and obliterative phlebitis 2,3. High IgG4 serum levels were detected in the majority of patients. However, this disease is seen in a wide range of ages but usually manifests itself in the fifth and sixth decades of life. Salivary gland disease and autoimmune pancreatitis are the two prevalent manifestations of IgG4-RD. Other affected systems and organs are the liver and biliary tract, lacrimal gland, Respiratory system, lymph nodes, and retroperitoneum4. Due to the indolent pattern of the disease with unfocused and vague symptoms, early diagnosing IgG4-RD is challenging. American College of Rheumatology (ACR) recommended the criteria for confirming IgG4-RD diagnosis. Using various clinical, paraclinical, histopathological, and radiological findings and considering the differential diagnoses make ACR criteria more comprehensive and reliable5.
There are few reports in head and brain involvements of IgG4-RD in the form of hypertrophic pachymeningitis, cranial nerve impairments, pituitary gland inflammatory lesions, orbital pseudotumor, pterygopalatine fossa infiltrations, and recently central nervous system (CNS) parenchymal involvement 6-9. Hypertrophic pachymeningitis characterized by dura matter inflammation in the periorbital areas, clivus, vestibular structures, brainstem, and spinal nerve roots and usually presents with focal neurological signs and symptoms 10. Cerebral Vein Thrombosis (CVT) may be associated with the local inflammatory setting near the pachymeningitis or the formed fibrosis tissue mechanical pressure2,11. Lymphoma, granulomatosis with polyangiitis (GPA), and neurosarcoidosis are the differential diagnoses of IgG4-RD pachymeningitis 12. CT-scan and MRI reveal linear thickening of the dura, and cerebrospinal fluids investigations are usually non-specific 13. However, a meningeal biopsy is invasive and risky; it is still the gold standard for the diagnosis. In this condition, searching for other organs’ silent involvements may be an appropriate guide to the diagnosis 14. Different stages and organ involvement in IgG4-RD respond dramatically to glucocorticoids. Other treatment options in patients who are resistant or unable to use glucocorticoids are Azathioprine, Mycophenolatemofetil, or Rituximab 4,5.
Here, we established IgG4-RD diagnosis in a young woman who presented with recurrent cerebral venous thrombosis (CVT), pachymeningitis, and an orbital mass.