ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies

Abstract

Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy.

Seronegative granulomatosis with polyangiitis presenting with multiple cranial nerve palsies

“…The CHCC 2012 defined this disease as necrotizing granulomatous inflammation and necrotizing vasculitis of small to medium‐sized vessels . The presence of anti‐neutrophil cytoplasmic antibody (ANCA), an autoimmune antibody, is adjunctive for its diagnosis …”

“…To diagnose GPA, histologic review via biopsy is decisive and imaging studies are helpful. Detection of either anti‐proteinase 3 (PR3)‐ANCA or myeloperoxidase (MPO)‐ANCA can support the diagnosis, but some cases are negative for ANCA …”

“…Without treatment, the expected patient survival is about 1 year after diagnosis and shorter for delayed diagnosis; however, the 5‐year survival rate increases to up to 80% with treatment. Therefore, early diagnosis with subsequent adequate treatment is critical for better prognosis …”

“…2 The presence of anti-neutrophil cytoplasmic antibody (ANCA), an autoimmune antibody, is adjunctive for its diagnosis. [3][4][5][6][7] GPA can manifest with variable symptoms and a wide range of severities in either an acute or chronic course, but renal and respiratory systems are commonly involved as the first clinical manifestations. In the kidneys, rapidly progressive glomerulonephritis (RPGN) occurs, leading to chronic renal failure, while the respiratory system manifestations involve the upper airways, trachea and lungs.…”

“…Detection of either anti-proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA can support the diagnosis, but some cases are negative for ANCA. 6,14,15 Immunosuppression is the treatment modality of this disease, in which corticosteroid and cytotoxic agents are predominantly used to manage the disease during the acute course. 16 Without treatment, the expected patient survival is about 1 year after diagnosis and shorter for delayed diagnosis; however, the 5-year survival rate increases to up to 80% with treatment.…”