RT Journal Article SR Electronic T1 An atypical presentation of granulomatosis with polyangiitis (GPA) with pituitary involvement JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP PA3678 DO 10.1183/13993003.congress-2015.PA3678 VO 46 IS suppl 59 A1 Muhammad Badar Ganaie A1 Ajit Thomas YR 2015 UL http://erj.ersjournals.com/content/46/suppl_59/PA3678.abstract AB Introduction: GPA or Wegener's is a rare multisystem vasculitis. We present a case that posed a diagnostic dilemma due to atypical presentation.Case report: 45 years old lady, presented with headache and no systemic symptoms. MRI head showed sellar lesion; differential was sarcoid, lymphoma and pituitary tumor. CSF showed non specific inflammation with negative cultures. Vasculitis screen including ANCA and ACE was negative. CT chest and PET-CT showed right upper lobe lesion, staged as T3,N2,M1 suspected lung cancer. Patient refused bronchoscopy. CT guided biopsy showed acute inflammation but no granuloma or malignancy. She underwent right upper and middle lobectomy. Histology revealed necrotising granulomas raising possibility of tuberculosis and was empirically treated.She presented a month later with hemoptysis and right eye ptosis. CT chest suggested recurrent malignancy with disease progression. MRI head showed progressive sellar mass. Repeat PR3 ANCA was positive. She was started on IV cyclophosphamide and steroids and was later switched to oral steroids and azathioprine with symptomatic and radiological resolution (see images).Conclusion: Neurological and pituitary involvement (especially anterior pituatory) in GPA is very rare. This case posed diagnostic uncertainty due to her atypical symptoms, initially negative ANCA and non-diagnostic histology. A high index of suspicion is necessary for establishing diagnosis.