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Granulomatous Disorder With Pulmonary and Renal Involvement: A Diagnostic and Therapeutic Dilemma
Merina Khan1, Nida Saleem1, Syed Nayer Mahmud1
1Nephrology, Shifa International Hospital Islamabad, Islamabad, PAK.
Abstract:
Granulomatosis with polyangiitis (GPA) can present with a wide array of clinical signs and symptoms; therefore, it should be differentiated from other mimicking clinicopathological entities. We report a case of a 66-year-old gentleman who was found to have a mediastinal mass and histopathological examination showed chronic necrotizing granulomatous inflammation. The patient was managed on lines of pulmonary tuberculosis for 12 months and remained in remission for two years. Later, workup showed cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA)-associated granuloma with marked renal impairment, which responded to immunosuppression. From this, we suggest that in a patient with radiological evidence of mediastinal mass, the remote possibility of GPA must be kept in mind.
Insights
Granulomatosis with polyangiitis (GPA) can mimic other conditions. This case highlights GPA presenting as a mediastinal mass, emphasizing the need for differential diagnosis in such presentations.
Area of Science:
- Pulmonology
- Rheumatology
- Pathology
Background:
- Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis.
- GPA diagnosis can be challenging due to diverse clinical presentations.
- Mediastinal masses require thorough differential diagnosis.
Observation:
- A 66-year-old male presented with a mediastinal mass.
- Initial diagnosis and management were for pulmonary tuberculosis.
- Histopathology revealed chronic necrotizing granulomatous inflammation.
Findings:
- The patient later developed cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA)-associated granuloma.
- Significant renal impairment was noted.
- Symptoms improved with immunosuppressive therapy.
Implications:
- GPA should be considered in patients with mediastinal masses.
- Early differentiation from infections like tuberculosis is crucial.
- c-ANCA testing aids in diagnosing GPA in complex cases.
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