In the International Journal of Rheumatic Diseases, researchers reported the case study of a patient with relapsed granulomatosis with polyangiitis (GPA), a form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).  

Patients with GPA can have a heterogenous presentation, with some patients suffering from severe, life-threatening disease (such as diffuse alveolar hemorrhage and/or glomerulonephritis). Despite the relative success of rituximab therapy, resistant and relapsing disease remains a substantial concern. 

A 46-year-old man presented with pyrexia, facial paralysis, and unknown discharge from his right ear. Cranial computed tomography (CT) demonstrated bilateral mastoiditis. His physicians started him on prednisolone and meropenem; however, they then noticed discharge from the opposite (left) ear. 

The patient also presented with dysuria with hematuria and pyuria; a working diagnosis of prostatitis was made and amikacin was prescribed. His serum creatinine levels were found to be at 1.1 mg/dL. Magnetic resonance imaging of the pelvis revealed a large prostatic abscess, measuring 110 × 58 mm. Drainage fluid analysis revealed necrosis with abundant neutrophils. 

Read more about AAV etiology 

A thoracic CT showed cavitating nodules in the lungs bilaterally. Proteinase-3-ANCA was elevated at 93 IU/L, and a renal biopsy demonstrated pauci-immune necrotizing glomerulonephritis. In light of these findings, the patient’s physicians initiated methylprednisolone and cyclophosphamide, which successfully induced remission. Maintenance with rituximab therapy was planned for twice a year. 

Following the second cycle of rituximab, the patient complained of severe headaches and altered consciousness. A repeat cranial CT showed 4 distinct abscess formations. Excision of the brain biopsy revealed necrotizing vasculitis with granuloma formation. Methylprednisolone was re-initiated, resolving the lesions a year later. The patient continues to be in remission 5 years after treatment. 

“Although rituximab is the first choice for maintenance treatment in most cases, the slow taper of corticosteroids and/or immunosuppressives would be preferable in resistant granulomatous involvement of GPA,” the authors wrote.


Ince B, Eroğlu E, Barburoğlu M, et al. Intracranial granuloma in a patient with granulomatosis with polyangiitis under rituximab maintenance: a case reportInt J Rheum Dis. Published online August 23, 2023. doi:10.1111/1756-185X.14887