IgG4-related disease with multiorgan involvement: a case-based review
Nikhil N Tarte1,2, Chandana Shilpa Ravipati3, Jose A Leon de la Rocha4
1Baton Rouge General Medical Center, Baton Rouge, LA, USA. ntarte2@uic.edu.
Rheumatology International
|April 21, 2021
Summary
This case study details a patient with extensive IgG4-related disease (IgG4-RD) affecting multiple organs. The 2019 ACR/EULAR criteria aided diagnosis, emphasizing early intervention for better outcomes.
Area of Science:
- Immunology
- Rheumatology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a complex immune-mediated condition characterized by IgG4-secreting plasma cell infiltration.
- It can affect multiple organs, leading to diverse clinical presentations and diagnostic challenges.
Observation:
- A patient presented with simultaneous involvement of kidneys, lymph nodes, bone marrow, lungs, liver, and small intestine.
- Biopsy confirmed bone marrow involvement, while imaging revealed abnormalities in other organs.
Findings:
- The case highlights the utility of the 2019 ACR/EULAR classification criteria, incorporating serologic, pathologic, and radiologic domains.
- The patient's IgG4-RD classification score was 48, meeting the criteria for diagnosis (≥20) with biopsy-proven involvement and no exclusion criteria.
Implications:
- This case underscores the importance of considering IgG4-RD in patients with extensive multi-organ involvement.
- Utilizing the comprehensive classification criteria can aid diagnosis, especially when biopsies are invasive or inaccessible.
- Early diagnosis and treatment are crucial to prevent irreversible organ damage and improve patient prognosis.
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