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Navigating the Diagnostic Maze: A Case Report of Immunoglobulin G4-Related Disease.

Authors :
Khan MA
Ullah N
Khan S
Ur Rehman W
Shah MA
Source :
Cureus [Cureus] 2024 Jul 14; Vol. 16 (7), pp. e64502. Date of Electronic Publication: 2024 Jul 14 (Print Publication: 2024).
Publication Year :
2024

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by significant infiltration of immunoglobulin G4 (IgG4)-positive plasma cells within affected tissues, with or without elevated serum IgG4 levels. The prevalence of IgG4-RD remains largely undetermined due to diagnostic challenges, as the condition is frequently unrecognized or misdiagnosed. This report describes a case of a 63-year-old man who was ultimately diagnosed with this rare condition after an extensive two-year period of elusive symptoms. Initially presenting with intermittent body pains and fluctuating fever, his condition progressively evolved to include severe right orbital swelling with marked tenderness and ecchymosis, recurrent non-tender nodules on his arm, and diminished vision. A detailed review of his medical history prompted the consideration of IgG4-RD, leading to the measurement of serum human IgG4 levels, which were found to be significantly elevated at 1504 mg/L (normal range: 39.2-864 mg/L). Following his diagnosis, treatment with glucocorticoids (0.6 mg/kg for one month) was initiated, resulting in a positive clinical response. This case emphasizes the critical importance of considering less common conditions in the differential diagnosis of patients presenting with complex, multi-system symptoms.<br />Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.<br /> (Copyright © 2024, Khan et al.)

Details

Language :
English
ISSN :
2168-8184
Volume :
16
Issue :
7
Database :
MEDLINE
Journal :
Cureus
Publication Type :
Academic Journal
Accession number :
39139309
Full Text :
https://doi.org/10.7759/cureus.64502