Bejerano C, Blanco R, González-Vela C, et al. Refractory polymyalgia rheumatica as presenting manifestation of large-vessel vasculitis associated to sarcoidosis. Successful response to adalimumab. Clin Exp Rheumatol. 30:(1 Suppl. 70):S94-S97
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; 19(5). DOI:10.1097/MCP.0b013e3283642ad0 · 2.96 Impact Factor
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ABSTRACT: Background Non-infectious aortitis is often an underdiagnosed and potentially serious clinical entity. Although giant cell arteritis (GCA) is considered to be the most common cause of aortitis, it may also be idiopathic or associated with other conditions. Clinical manfiestations are frequently nonspecifc leading to inapropriate delay to the diagnosis. Methods Multicenter study of three hospitals of patients diagnosed as having aortitis by F18-FDG PET. Results Twelve patients (8 women/4 men) with a mean age of 68.4±12.7 years were diagnosed with aortitis by F18-FDG PET (TABLE). The median [interquartile range] of delay to the diagnosis was 12 [2-20] months. Aortitis was idiopathic (5 cases), associated with biopsy-proven GCA (n=4), Sarcoidosis (n=1), Ulcerative Colitis (n=1), and Takayasu arteritis (n=1). The most frequent symptoms were: A) Polymyalgia rheumatica (PMR) in 8 cases (67%), that was atypical in 5 of them. B) Fever and/or general syndrome in 6 cases (50%). And C) Severe low back pain in 4 cases (33%). Laboratory data disclosed anemia in all cases and high ESR in most cases (78.1±38.3 mm/1st hour). PET showed involvement of A) the whole aorta, thoracic aorta and/or supra-aortic vessels in 91% and B) involvement of arteries of the lower extremities in 50%. Conclusions F18 FDG-PET is a useful tool to confirm the diagnosis of aortitis. Atypical PMR, non-specific general syndrome, and/or unexplained severe low back pain along with anemia and elevated ESR may be red flags to suspect this condition. Disclosure of Interest None DeclaredAnnals of the Rheumatic Diseases 01/2014; 71(Suppl 3):708-709. DOI:10.1136/annrheumdis-2012-eular.1241 · 10.38 Impact Factor