011. GRANULOMATOSIS WITH POLYANGIITIS PRESENTING AS AUTOIMMUNE PANCREATITIS

Abstract

Background: This 52 year old female presented in January 2016 to the hepatobiliary surgical team with anorexia, weight loss, jaundice and elevated liver function tests. MRCP showed dilatation of the biliary and pancreatic ducts with subsequent ERCP confirmation of narrowing of the common bile duct and negative for any malignancy. The common bile duct was stented and patient was diagnosed and managed as a probable autoimmune pancreatitis with oral steroid with good effect. Weaning of steroid proved difficult due to return of symptomatology and a diagnosis of steroid induced diabetes was also made. In August 2016, progressive shortness of breath on exertion, non-productive cough and right sided pleuritic chest pain resulted in re-presentation to hospital. Chest X-ray showed a right sided pleural effusion with collapse and consolidation and antibiotic therapy was commenced. Due to no real improvement with antibiotic therapy a CT scan of the chest was performed showing similar findings to the chest X-ray, but in addition showed a number of small lung nodules and a differential of metastasis was considered. Histology from CT guided biopsy showed evidence of necrotizing granulomatous inflammation. ANCA screening subsequently showed a C-ANCA of 80 and a PR3 >8 suggesting a diagnosis of granulomatosis with polyangiitis. In addition to chest pathology a urinalysis showed+++ blood and+++ protein with normal renal function in keeping with probable renal involvement. Results: On review in rheumatology clinic the patient had features suggesting pulmonary and renal involvement as well as bilateral episcleritis, peripheral neuropathy, vasculitic rash on lower limbs and arthralgia/myalgia. With recent investigation results available, treatment was initiated with oral prednisolone 60mg and patient counselled for treatment with intravenous cyclophosphamide. Steroid therapy made a dramatic improvement to symptoms with rapid fall of CRP from 250mg/L to normal within a few weeks. Unfortunately as treatment with cyclophosphamide got under way, renal function also deteriorated with estimated glomerular filtration rate falling to 8ml/min and creatinine climbing to 482 μmol/L. Under the careful watch of the renal physicians, cyclophosphamide was continued and after 2 weeks, renal function slowly improved and plasma exchange was narrowly avoided. Cyclophosphamide and reducing steroid therapy is ongoing and the patient is progressing well. Conclusion: This case is interesting as although the respiratory and renal involvement are classical presentations of granulomatosis with polyangiitis, in retrospect the admission to hospital 6 months prior with autoimmune pancreatitis was likely an unrecognized feature of this patient's evolving ANCA associated vasculitis. Investigation of pancreatitis leading to a diagnosis of granulomatosis with polyangiitis is rare but is recorded in the literature with a number of case reports. It highlights the need to be vigilant for underlying ANCA-associated vasculidities in patients presenting with pancreatitis of unclear aetiology.