Wegener's Granulomatosis ResearchLimited Wegener's granulomatosis presenting as lung nodules in a patient with rheumatoid arthritis: a case report.Primary Author: Sushma PaiPrimary Author: Date Published: 2008 12 24Abstract: ABSTRACT: BACKGROUND: Rheumatoid arthritis has varied pleuroparenchymal manifestations. Wegener's granulomatosis can develop in an established case of rheumatoid arthritis and this association although previously reported is very rare. CASE PRESENTATION: A 60-year-old lady had been diagnosed with rheumatoid arthritis on the basis of her clinical symptoms and serological tests which were positive RA factor and anti-CCP antibodies. Her rheumatoid arthritis activity had been mild and well controlled with hydroxychloroquine and low dose prednisone. She presented with a productive cough and right-sided pleuritic chest pain. CT scan of the chest showed three lung nodules with increased uptake on PET CT scan, raising concerns for an inflammatory or malignant process. The differential diagnosis included rheumatoid nodules, infections or malignancy. A CT-guided needle biopsy of the largest nodule was undertaken which showed vasculitis typical of Wegener's granulomatosis. Stains and cultures of the biopsy specimen were negative for bacteria, fungi and acid fast bacilli. A panel of serological tests for vasculitis were checked and showed elevated titers of cANCA and anti-proteinase 3 antibodies. Urine analysis and CT scan of paranasal sinuses was normal. Since the upper respiratory tract and the kidneys were spared a diagnosis of limited Wegener's granulomatosis affecting only the lungs was made. Due to the toxicity of cyclophosphamide, her relatively mild disease sparing the kidneys and the underlying rheumatoid arthritis, weekly methotrexate was started and low dose prednisone was continued. She had marked symptomatic improvement and complete resolution of the nodules was documented on subsequent imaging. CONCLUSION: Wegener's granulomatosis developing in a patient with rheumatoid arthritis is very rare but should be considered as it warrants a different and possibly more aggressive treatment approach.
[Cyclophosphamide versus bolus in Wegener's granulomatosis and other ANCA-related vasculitides : Advantages and disadvantages.]Primary Author: E Reinhold-KellerPrimary Author: Rheumaklinik Bad Bramstedt & Internistisch-rheumatologische Gemeinschaftspraxis Hamburg, Oskar-Alexander-Str. 26, 24576, Bad Bramstedt, Deutschland, reinhold-keller@rheuma-spezialisten.de.Date Published: 2008 12 18Abstract: Cyclophosphamide remains the therapy of choice in severe ANCA positive vasculitis. In order to avoid the considerable side effects of high cumulative doses sometimes manifesting themselves years later, induction therapy with a low cyclophosphamide dose and an early switch to cyclophosphamide-free maintenance regimens should be aimed for. This can be achieved by an induction therapy of 3--6 months of intravenous pulse therapy (pCYC) or oral therapy (oCYC). The metaanalysis of therapeutic trials in ANCA vasculitis demonstrates a slightly higher induction rate of remission, but an increased relapse rate of pCYC compared to oCYC. The incidence of adverse events seems to be higher in oCYC compared to pCYC. The results of the first controlled prospective multicenter trial (CYCLOPS) directly comparing pCYC and oCYC treatment of ANCA-positive vasculitis are eagerly awaited. It will be important to extend the follow-up over several years in order to reach a valid estimate of the incidence of secondary malignancies due to CYC therapy.
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