Refractory polymyalgia rheumatica as presenting manifestation of large-vessel vasculitis associated to sarcoidosis. Successful response to adalimumab.
ABSTRACT Sarcoidosis may present with musculoskeletal features or mimic rheumatic diseases. We report on a patient who had been initially diagnosed as having polymyalgia rheumatica. Because of refractory disease associated to atypical features such as severe inflammatory low back pain, dull and achy pain in the thighs, claudication of the lower limbs and bad response to corticosteroids and methotrexate (MTX), an 18F-fluorodeoxyglucosepositron emission tomography with CT (FDG PET/CT) was performed. This technique disclosed data suggestive of arteritis of large vessels involving the ascending, arch and descending aorta as well as high FDG uptake in the femoral and posterior tibial arteries of both lower extremities. Also, increased FDG uptake was observed in the right paratracheal, retrotracheal, subcarinal, gastrohepatic ligament, coeliac and right renal hilar lymph nodes. Four lymph nodes, taken during mediastinoscopy, confirmed a diagnosis of sarcoidosis. Treatment with adalimumab (40 mg every 2 weeks subcutaneously) along with prednisone and MTX was initiated yielding progressive improvement of symptoms and normalisation of laboratory abnormalities. Five months after the onset of adalimumab a new FDG PET/CT showed complete absence of uptake of lymph nodes as well as decrease of vascular FDG uptake. To our knowledge, this is the first patient treated with adalimumab because of a large-vessel vasculitis in the setting of sarcoidosis refractory to conventional therapy. This case reinforces the claim that sarcoidosis should be considered a diagnostic challenge in the assessment of patients presenting with inflammatory musculoskeletal symptoms.
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ABSTRACT: Vasculitis syndromes are relative rare conditions but can cause significant mortality and morbidity if not treated adequately. Recent advances in immunosuppressant therapy have radically changed the course of these diseases. However, the standard therapy is not always well tolerated by patients, and some cases are refractory to treatment. New therapeutic possibilities have emerged with the use of so-called "biologics," a new class of genetically engineered drugs used for inflammatory rheumatic diseases, including rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis. In the present review, summarized are the most recent data on the efficacy and safety of biologics in the treatment of vasculitis syndromes that cannot be treated with standard therapy.Biologics: Targets & Therapy 10/2012; 6:371-8. DOI:10.2147/BTT.S37537
Article: Nonsteroidal therapy of sarcoidosis.[Show abstract] [Hide abstract]
ABSTRACT: None of the medications used in clinical practice to treat sarcoidosis have been approved by the regulatory authorities. Understanding how to use disease-modifying antisarcoid drugs, however, is essential for physicians treating patients with sarcoidosis. This review summarizes the recent studies of medications used for sarcoidosis with a focus on nonsteroidal therapies. Studies from 2006 to 2013 were considered for review to update clinicians on the most relevant literature published over the last few years. Several recently published pieces of evidence have helped expand our ability to more appropriately sequence second-line and third-line therapies for sarcoidosis. For instance, methotrexate and azathioprine may be useful and well tolerated medications as second-line treatment. Mycophenolate mofetil might have a role in neurosarcoidosis. TNF-α blockers and other biologics seem to be well tolerated medications for the most severely affected patients. Corticosteroids remain the first-line therapy for sarcoidosis as many patients never require treatment or only necessitate a short treatment duration. Second-line and third-line therapies described in this article should be used in patients with progressive or refractory disease or when life-threatening complications are evident at the time of presentation.Current opinion in pulmonary medicine 07/2013; DOI:10.1097/MCP.0b013e3283642ad0 · 2.96 Impact Factor
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ABSTRACT: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are two closely related diseases in people aged 50 years and older, which are more frequently observed in Western countries. Despite being common entities, concern still exists about the epidemiology, pathogenesis, and diagnosis of both entities. New imaging techniques, such as 18 fluorodeoxyglucose-positron emission tomography, have proved to be useful in detecting large-vessel involvement in GCA. Corticosteroids are the cornerstone of the therapy in GCA and PMR. Relapses are frequent in these conditions. Unlike methotrexate and tumor necrosis factor-α antagonists, anti-interleukin-6 receptor therapy appears to be useful in patients with GCA and PMR who are refractory to corticosteroids. This review summarizes recent studies on GCA and PMR.Current Rheumatology Reports 02/2015; 17(2):480. DOI:10.1007/s11926-014-0480-1 · 2.45 Impact Factor