Anti-neutrophil cytoplasmic antibody (ANCA) associated granulomatous vasculitis presenting as post-menopausal bleeding and abnormal cervix – a case study and literature review.

Royal Preston Hospital UK

Anti-neutrophil cytoplasmic antibody (ANCA) associated granulomatous vasculitis, previously known as Wegener’s granulomatosis, is a rare multi-organ autoimmune necrotising vasculitis of unknown aetiology. It is characterised by granulomatous inflammation, vasculitis of small-to-medium vessels and elevated cytoplasmic ANCA. It most commonly affects the upper respiratory tract, lungs and kidneys. Its involvement with the cervix is extremely rare; to date there have been only been five published cases in the literature.

In this case we present a 66 year old female with ANCA associated granulomatous vasculitis of the cervix. This patient initially presented in 2006 with upper respiratory tract symptoms, fever and renal failure. A renal biopsy later confirmed the diagnosis of ANCA-associated granulomatous vasculitis. The patient was managed with prednisolone and cyclophosphamide, later changing to azothiaprine. The patient remained in remission for six years. She presented in 2012 to her GP with a 12-month history of minimal vaginal bleeding and thrush. No relapse of her previous vasculitis symptoms was suspected. The patient was seen in a gynaecology clinic where speculum exam revealed an irregular appearance of the cervix; this was viewed in the colposcopy clinic demonsrating an irregular ‘suspicious’ cervix with contact bleeding. Cervical biopsies were taken reporting granulation tissue, giant cell reaction with vessel vasculitis but no neoplasia. The patient also underwent hysteroscopy, endometrial biopsy and further cervical biopsies that showed a normal endometrial cavity and a persistent abnormality of the cervix. On review, CRP and ANCA, when trended over the past year, showed an upward trend indicating active disease; however renal function remained stable. The patient is awaiting a PET-CT scan to identify any other areas of active disease. This will aid in determining possible management plans, including increasing the dose of azothiaprine, restarting cyclophosphamide and subsequent review of her genital tract.