Simultaneous presentation of granulomatosis with polyangiitis (GPA) and immunoglobulin G4-related disease (IgG4-RD). Leaving an open question: widening the spectrum of a single disease or real overlap?
| Autor: | João Fernandes-Serodio, Manel Solé, Maria T Boncoraglio, Juan M Corral-Molina, José Hernández-Rodríguez, Sergio Prieto-González |
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| Rok vydání: | 2020 |
| Předmět: |
Male
Pathology medicine.medical_specialty ANCA-Associated Vasculitis macromolecular substances Disease Antibodies Antineutrophil Cytoplasmic Glomerulonephritis Immunoglobulin g4 parasitic diseases Humans Medicine skin and connective tissue diseases integumentary system business.industry fungi Granulomatosis with Polyangiitis Middle Aged medicine.disease Prednisone Organ involvement Immunoglobulin G4-Related Disease Presentation (obstetrics) Rituximab business Vasculitis Granulomatosis with polyangiitis |
| Zdroj: | Modern Rheumatology Case Reports. 5:108-112 |
| ISSN: | 2472-5625 |
| DOI: | 10.1080/24725625.2020.1782028 |
| Popis: | Because of a similar organ involvement and histopathological features, IgG4-related disease (IgG4-RD) may mimic some forms of granulomatosis with polyangiitis (GPA). However, several cases of clear coexistence or overlap of both diseases have been reported. We describe a case of a 47-year-old man presenting with a renal mass and a nasal crusting showing histopathological features of IgG4-RD in both territories. Cytoplasmic/proteinase 3 (PR3) antineutrophil cytoplasmic antibodies (ANCA) were positive and the patient subsequently developed kidney failure and nephritic syndrome that led to a renal biopsy re-evaluation revealing changes compatible with segmental necrotising glomerulonephritis and GPA. Remission induction therapy with prednisone and rituximab was started and clinical and laboratory parameters returned to normal. After administering a maintenance regimen based in rituximab 500 mg every six month the patient remained asymptomatic during 4 years of follow-up and free of prednisone the last 18 months. Although coexistence or overlap of GPA and IgG4-RD may be established in some clinical scenarios, the possibility of widening the spectrum of a single disease is also postulated. |
| Databáze: | OpenAIRE |
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