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Granulomatosis with polyangiitis: The trigger cannot be long hidden
Shameek Gayen1, Diana Zhang1, Eliza Sternlicht1
1Department of Medicine, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts, USA.
Granulomatosis with polyangiitis (GPA) is a rare vasculitis. This case highlights cytomegalovirus infection as a potential trigger in an undiagnosed immunocompromised patient, emphasizing diagnostic challenges.
Area of Science:
- Immunology
- Nephrology
- Rheumatology
Background:
- Granulomatosis with polyangiitis (GPA) is a systemic vasculitis affecting multiple organs, notably the respiratory tract and kidneys.
- Diagnosis typically involves clinical suspicion, elevated antineutrophil cytoplasmic antibodies (ANCA), and organ biopsy confirmation.
- Viral infections are implicated as potential triggers for GPA development.
Purpose of the Study:
- To report a rare case of GPA.
- To investigate the association between cytomegalovirus (CMV) infection and GPA onset.
- To highlight diagnostic considerations in immunocompromised patients.
Main Methods:
- Case report detailing clinical presentation and diagnostic workup.
- Review of patient's medical history for underlying immunocompromised status.
- Serological and biopsy confirmation of GPA and CMV infection.
Main Results:
- The patient presented with clinical features suggestive of GPA.
- Elevated ANCA titers and positive biopsy confirmed GPA.
- Concurrent cytomegalovirus infection was identified in a patient with an undiagnosed immunocompromised state.
Conclusions:
- Cytomegalovirus infection can precipitate GPA in individuals with previously unrecognized immunocompromise.
- This case underscores the importance of considering viral triggers in GPA pathogenesis.
- Early identification of underlying immune deficiencies is crucial for managing GPA.
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