title: 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.
authors.affiliation: Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IFIMAV, Santander, Spain. cbejerano@hotmail.com
authors.forename: Carmen
authors.initials: C
authors.lastname: Bejerano
authors.forename: Ricardo
authors.initials: R
authors.lastname: Blanco
authors.forename: Carmen
authors.initials: C
authors.lastname: González-Vela
authors.forename: Ramón
authors.initials: R
authors.lastname: Agüero
authors.forename: José M
authors.initials: JM
authors.lastname: Carril
authors.forename: Miguel A
authors.initials: MA
authors.lastname: González-Gay
mesh_terms: D000068879:Adalimumab; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001706:Biopsy; D004359:Drug Therapy, Combination; D019788:Fluorodeoxyglucose F18; D006801:Humans; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D064847:Multimodal Imaging; D011111:Polymyalgia Rheumatica / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D049268:Positron-Emission Tomography; D011237:Predictive Value of Tests; D011241:Prednisone / Q000627:therapeutic use; D019275:Radiopharmaceuticals; D012507:Sarcoidosis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D051598:Whole Body Imaging
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D018501:Antirheumatic Agents; D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18; D000068879:Adalimumab; D011241:Prednisone; D008727:Methotrexate