036 Pulmonary rheumatoid vasculitis sans arthritis: a rare case

Abstract

Background: Pulmonary rheumatoid vasculitis as a primary presentation of rheumatoid arthritis is very rare, especially in the complete absence of arthritis. Methods: We are presenting a case of a female who presented with multiple pulmonary nodules, and pulmonary rheumatoid vasculitis with no evidence of arthritis. Results: A 74 year old female attended her GP in August 2016 with a four week history of low grade haemoptysis and pleurisy as well as raised inflammatory markers. Relevant past medical history included a right total hip replacement (right) in 2012, an asymptomatic right median nerve neuropathy in 2013, and generalised osteoarthritis. An urgent CT Thorax was arranged which revealed multiple basal nodules. In order to characterise the lesions, a whole body PET-CT was organised, which demonstrated weakly positive basal nodules with some cavitation but no other significant findings save for nonhomeogenous tracer uptake in the liver. At this stage the differential diagnoses included vasculitis, infection and metastatic malignancy. ANCA was negative but RF was strongly positive. At the time the lesion was not amenable to percutaneous biopsy and she declined anything more invasive, but remained under regular surveillance. In February 2017, she presented acutely with large volume haemoptysis. CT Thorax demonstrated progression of the lung nodules. At this stage she was able to have a CT guided biopsy but this unfortunately proved inconclusive; however the findings were sufficient enough to make malignancy unlikely. After discussion at Lung MDT the probable diagnosis was thought to be a low grade ANCA negative vasculitis. A VATS biopsy was arranged for June 2017 and wedge resection of the right lower lobe was performed. Histology suggested a rheumatoid vasculitis and AntiCCP returned confirmatory. She was commenced on steroids by July. On the 15th of August 2017 she was admitted with acute shortness of breath. CT Angiogram revealed a pulmonary embolism, and Apixaban was started. As of September 2017 there has been radiological regression of the lung nodules, coupled with symptomatic improvement and pulmonary function, with a FEV1/FVC ratio of 82% and FVC of 100% when compared to age and sex matched controls. Conclusion: Rheumatoid vasculitis is traditionally seen in longstanding, active rheumatoid arthritis. It is unusual to see it as the primary presenting feature. In this patient, the histology prompted Anti-CCP antibody testing which returned strongly positive. The initial suspicion was an ANCA-negative vasculitis, but the histology was consistent with rheumatoid vasculitis. There are few case reports in the literature documenting pulmonary rheumatoid vasculitis predating inflammatory arthritis, of which the majority developed arthritis very soon after the initial presentation. Therefore, there is a case for starting DMARD therapy promptly.