IgG4-related disease presenting with multiorgan involvement. 2022

Roberto Tellez, and Brittany Bromfield, and Hayden Basinger, and Adam Slivka
Internal Medicine, UPMC, Pittsburgh, Pennsylvania, USA.

Immunoglobulin G4-related disease (IgG4-RD) is a rare fibroinflammatory immune-mediated condition which can affect multiple organ systems and form mass-like lesions. Initial presentation can mimic other diseases such as pancreatic malignancy when there is pancreatic involvement or tuberculosis (TB) when there are pulmonary lesions or hypertrophic pachymeningitis (HP). Here, we report a novel case of IgG4-RD presenting as bilateral subdural haematomas with additional findings. Our patient is a male who presented with headaches and blurred vision. Physical examination showed disconjugate gaze with a fixed pupil. Trauma survey radiologic imaging revealed a pancreatic mass concerning for malignancy. Subsequent workup found hypophysitis with optic chiasm compression and hypopituitarism, mediastinal lymphadenopathy and HP. Laboratory values showed an elevated serum IgG4 level and latent TB. Our case adds to the existing IgG4-RD literature by highlighting a unique presentation. It is important to maintain it on the differential diagnosis especially in multisystemic presentations with competing diagnoses.

UI MeSH Term Description Entries
D006984 Hypertrophy General increase in bulk of a part or organ due to CELL ENLARGEMENT and accumulation of FLUIDS AND SECRETIONS, not due to tumor formation, nor to an increase in the number of cells (HYPERPLASIA). Hypertrophies
D007074 Immunoglobulin G The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B. Gamma Globulin, 7S,IgG,IgG Antibody,Allerglobuline,IgG(T),IgG1,IgG2,IgG2A,IgG2B,IgG3,IgG4,Immunoglobulin GT,Polyglobin,7S Gamma Globulin,Antibody, IgG,GT, Immunoglobulin
D008297 Male Males
D008581 Meningitis Inflammation of the coverings of the brain and/or spinal cord, which consist of the PIA MATER; ARACHNOID; and DURA MATER. Infections (viral, bacterial, and fungal) are the most common causes of this condition, but subarachnoid hemorrhage (HEMORRHAGES, SUBARACHNOID), chemical irritation (chemical MENINGITIS), granulomatous conditions, neoplastic conditions (CARCINOMATOUS MENINGITIS), and other inflammatory conditions may produce this syndrome. (From Joynt, Clinical Neurology, 1994, Ch24, p6) Pachymeningitis,Meningitides,Pachymeningitides
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000072281 Lymphadenopathy Disease of LYMPH NODES which are abnormal in size, number or consistency. Adenopathy,Adenopathies,Lymphadenopathies
D000077733 Immunoglobulin G4-Related Disease A spectrum of systemic autoimmune diseases in which IMMUNOGLOBULIN G4 plays a pathophysiologic role. It can affect multiple organs in highly variable presentations, characterized by inflammatory lesions composed of IgG4-positive PLASMA CELLS, further infiltrated by T helper cells (T-LYMPHOCYTES, HELPER-INDUCER) when linked to progressive FIBROSIS and eventual organ damage. IgG4-Related Kidney Disease,IgG4-Related Sclerosing Disease,Immunoglobulin G4-Related Kidney Disease,Immunoglobulin G4-Related Sclerosing Disease,IgG4 Related Systemic Disease,IgG4-Associated Autoimmune Disease,IgG4-RD,IgG4-Related Disease,Autoimmune Disease, IgG4-Associated,IgG4 Associated Autoimmune Disease,IgG4 Related Disease,IgG4 Related Kidney Disease,IgG4 Related Sclerosing Disease,IgG4-Associated Autoimmune Diseases,IgG4-Related Diseases,IgG4-Related Kidney Diseases,IgG4-Related Sclerosing Diseases,Immunoglobulin G4 Related Disease,Immunoglobulin G4 Related Kidney Disease,Immunoglobulin G4 Related Sclerosing Disease,Immunoglobulin G4-Related Diseases,Kidney Disease, IgG4-Related,Sclerosing Disease, IgG4-Related

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