IgG4 disease with multiorgan involvement: a case report

IgG4-related disease (IgG4RD) is a rare fibroinflammatory disease with multiorgan involvement. It presents insidiously over several years and can be a diagnostic enigma. Delays in diagnosis occur due to failure to consider IgG4 as a differential diagnosis, atypical presentations, and an insidious cl...

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Bibliographic Details
Published in:Journal of medical case reports 2024-02, Vol.18 (1), p.115-115, Article 115
Main Authors: Vidanapathirana, M N, Wijayaratne, D
Format: Article
Language:English
Subjects:
Abdomen
Biopsy
Case Report
Development and progression
Health aspects
Histology
Histopathology
IgG4 disease
Immunoglobulin G
Inflammatory diseases
Kidneys
Lung diseases
Lungs
Lymphoma
Lymphomas
Medical diagnosis
Medical imaging
Mononeuritis multiplex
Nervous system
Neurological
Peripheral neuropathy
Pulmonary
Pulmonary function tests
Renal
Rheumatism
Rheumatology
Sarcoidosis
Serology
Tuberculosis
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Summary:IgG4-related disease (IgG4RD) is a rare fibroinflammatory disease with multiorgan involvement. It presents insidiously over several years and can be a diagnostic enigma. Delays in diagnosis occur due to failure to consider IgG4 as a differential diagnosis, atypical presentations, and an insidious clinical course. We report the case of a 70-year-old Sri Lankan man with pulmonary, renal, and neurological involvement of IgG4-related disease. Clinical manifestations evolved over a 4-year period and included exertional shortness of breath and dysesthesia of extremities. The diagnosis was established with clinical, radiological, and pathological criteria laid down by The American College of Rheumatology/European League Against Rheumatism in 2019. Following diagnosis, the patient was started on oral steroids, with rapid improvement of his respiratory and neurological symptoms. He is currently under follow-up and will be monitored with clinical and radiological parameters, complement levels, and lung function tests. This case outlines the presentation of a patient with IgG4-related disease with concurrent involvement of three uncommon sites. It highlights methods of diagnostic deduction by considering the clinical course of illness, imaging, and histopathology. It also describes evolving associations of IgG4-related disease with tuberculosis and lymphomas, which bear important diagnostic and therapeutic considerations.
ISSN:1752-1947
1752-1947
DOI:10.1186/s13256-024-04418-1