Espinoza-Gómez F, García CÁI, Garibay MR, Nava-Zavala AH

Language: Spanish
References: 11
Page: 38-43
PDF size: 234.91 Kb.

ABSTRACT

Granulomatosis with polyangiitis (GPA) is a type of vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA), with the main antigen being proteinase-3 (cANCA- PR3). GPA affects small- and medium-sized vessels of the respiratory tract and kidneys, leading to varying degrees of glomerulonephritis and pneumonitis. The occurrence of nephrotic syndrome (NS) and acute respiratory distress syndrome (ARDS) in association with GPA is extremely rare and poses a diagnostic and therapeutic challenge.
We report the case of a patient with type 2 diabetes mellitus (DM2) who developed NS with hematuria and rapidly progressive renal injury, along with ARDS and pulmonary nodules. Despite treatment with hemodialysis, mechanical ventilation, and intravenous methylprednisolone, the clinical course was fatal after three weeks. A positive cANCA-PR3 result confirmed the diagnosis of GPA.
The development of NS with hematuria and rapidly progressive renal failure in patients with diabetes should raise suspicion for vasculitis, such as GPA. Similarly, the onset of ARDS in patients with GPA-related lung disease is highly unusual. Therefore, the combination of GPA, DM2, NS with rapidly progressive renal injury, and ARDS represents a major diagnostic and therapeutic challenge that should be considered when managing diabetic patients with atypical nephropathy.

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