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.