Granulomatosis polyangiitis (Wegener's granulomatosis) is an ANCA-associated necrotising vasculitis. The disease involves upper respiratory tract, the lungs and kidneys but central nervous system (CNS) involvement is 1-5%.
A 40-year-old male patient was admitted to the hospital with joint pain, rash, aphthous lesions. The skin biopsy from the lesion showed leukocytoclastic vasculitis. The patient had c-ANCA positive and was diagnosed granulomatosis polyangiitis. He was treated with a pulse steroid and cyclophosphamide. Before the 5th session of therapy, the patient developed hemoptysis and hematuria. Thorax CT (computarized tomography) showed a diffuse alveolar hemorrhage and hence plasmapheresis and IVIG (intravenous immunoglobulin) were added to the treatment. Two days after IVIG, the patient developed globe vesical, headache and respiratory arrest. MR (magnetic resonance) showed CNS involvement. The patient was treated with a pulse steroid, but did not respond to therapy and died after 5 months since establishing the diagnosis.
More studies are needed to identify effective treatment and course of disease for patients with central nervous system involvement.