Adult-Onset Still’s Disease
Özet
Adult-onset Still’s Disease (AOSD) is a rare systemic autoinflammatory disease. While its exact cause is unknown, genetic predisposition, infections, and inflammatory processes may play a role. Yamaguchi and Fautrel criteria are used for diagnosis. Common symptoms include high fever, joint pain, skin rashes, leukocytosis, and elevated ferritin levels.
Treatment involves corticosteroids and disease-modifying antirheumatic drugs (DMARDs). In refractory cases, biologic DMARDs (IL-1 and IL-6 antagonists) can be effective.
Cardiac complications can lead to severe morbidity and mortality. The most common are pericarditis, myocarditis, endocarditis, and pulmonary arterial hypertension. Myocarditis and endocarditis are rare but potentially fatal.
Management focuses on reducing systemic inflammation with corticosteroids and DMARDs. In severe cases, biologic agents or intensive care interventions may be required. Cardiac complications in AOSD indicate disease severity and necessitate early detection for better outcomes.
Erişin Başlangıçlı Still Hastalığı (AOSD), nadir görülen sistemik bir otoinflamatuar hastalıktır. Nedeni tam olarak bilinmemekle birlikte genetik yatkınlık, enfeksiyonlar ve inflamatuar süreçler rol oynayabilir. Yamaguchi ve Fautrel kriterleri teşhis için kullanılır. Yaygın belirtiler yüksek ateş, eklem ağrısı, deri döküntüleri, lökositoz ve yüksek ferritin seviyeleridir.
Tedavide kortikosteroidler ve hastalık modifiye edici antiromatizmal ilaçlar (DMARDs) kullanılır. Dirençli vakalarda biyolojik DMARD’lar (IL-1 ve IL-6 antagonistleri) etkili olabilir.
Kalp komplikasyonları, ciddi morbidite ve mortaliteye yol açabilir. En sık görülenleri perikardit, miyokardit, endokardit ve pulmoner arteriyel hipertansiyondur. Miyokardit ve endokardit nadir ancak ölümcül olabilir.
Tedavi, sistemik inflamasyonu baskılamak için kortikosteroidler ve DMARD’larla başlar. Şiddetli durumlarda biyolojik ajanlar veya yoğun bakım müdahaleleri gerekebilir. AOSD hastalarında kalp komplikasyonları, hastalığın ciddiyetini belirleyen önemli bir faktördür ve erken teşhis hayati önem taşır.
Referanslar
Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still’s disease. J Autoimmun 2018;93:24–36. https://doi.org/10.1016/j.jaut.2018.07.018
Wang MY, Jia JC, Yang CD, Hu QY. Pathogenesis, disease course, and prognosis of adult-onset Still's disease: an update and review. ChinMed J (Engl) 2019;132:2856–2864
Kawaguchi H, Tsuboi H, Yagishita M, et al. Severe adult-onset Still disease with constrictive pericarditis and pleuritis that was successfully treated with tocilizumab in addition to corticosteroids and cyclosporin A. Intern Med. 2018;57:1033–1038
M. Yamaguchi, A. Ohta, T. Tsunematsu, R. Kasukawa, Y. Mizushima, H. Kashiwagi, et al., Preliminary criteria for classification of adult Still’sdisease, J Rheumatol. 19 (1992) 424–430.
B. Fautrel, E. Zing, J.-L. Golmard, G. Le Moel, A. Bissery, C. Rioux, et al., Proposal for a new set of classification criteria for adult-onset still disease, Medicine (Baltim.) 81 (2002) 194–200.
P. Sfriso, R. Priori, G. Valesini, S. Rossi, C.M. Montecucco, A. D’Ascanio, et al., Adult-onset Still’s disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients, Clin Rheumatol. 35 (2016) 1683–1689.
M. Gerfaud-Valentin, Y. Jamilloux, J. Iwaz, P. S`eve, Adult-onset Still’s disease, Autoimmun Rev. 13 (2014) 708–722.
S. Mitrovic, B. Fautrel. Complications of adult-onset Still’s disease and their management, Expert Rev. Clin. Immunol. 2018;14 (5);351–365.
M. Gerfaud-Valentin, P. S`eve, J. Iwaz, A. Gagnard, C. Broussolle, I. Durieu, et al., Myocarditis in adult-onset still disease, Medicine (Baltim.) 93 (2014) 280–289.
I. Ben Ghorbel, M. Lamloum, M. Miled, N. Aoun, M.-H. Houman, J. Pouchot. Adult-onset Still’s disease revealed by a pericardial tamponade: report of two cases. Rev. Med. Interne 27 (2006) 546–549.
B.M. Ertugrul, G. Uyar, B. Ozturk, S. Sakarya, A rare presentation of endocarditis in adult-onset Still’s disease in diagnosis of fever of unknown origin, J. Rheumatol. 39 (2012) 198–199.
Bodard Q., Langlois V., Guilpain P. Cardiac involvement in adult-onset Still’s disease: Manifestations, treatments and outcomes in a retrospective study of 28 patients. J Autoimmun. 2021;116:102541.
P. Sfriso, R. Priori, G. Valesini, S. Rossi, C.M. Montecucco, A. D’Ascanio, et al., Adult-onset Still’s disease: an Italian multicentre retrospective observational study of manifestations and treatments in 245 patients, Clin. Rheumatol. 35 (2016) 1683–1689.
F. Ortiz-Sanjuan, ´ R. Blanco, L. Riancho-Zarrabeitia, S. Castaneda, ˜ A. Oliv´e, A. Riveros, et al., Efficacy of anakinra in refractory adult-onset Still’s disease, Medicine (Baltim.) 94 (39) (2015).
F. Ortiz-Sanjuan, ´ R. Blanco, V. Calvo-Rio, J. Narvaez, E. Rubio Romero, A. Oliv´e, et al., Efficacy of tocilizumab in conventional treatment-refractory adult-onset Still’s disease: multicenter retrospective open-label study of thirty-four patients: tocilizumab in AOSD refractory to standard treatment, Arthritis Rheum. 66 (2014) 1659–1665
R. Giacomelli, P. Ruscitti, Y. Shoenfeld, A comprehensive review on adult onset Still’s disease, J. Autoimmun. 93 (2018) 24–36.
Pouchot J., Sampalis J.S., Beaudet F. Adult Still’s disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 1991;70:118–136.
Gracia-Ramos A.E., Contreras-Ortiz J.A. Myocarditis in adult-onset Still’s disease: case-based review. Clin Rheumatol. 2020;39:933–947.
Fautrel B. Adult-onset Still disease. Best Pract Res Clin Rheumatol. 2008; 22(5):773-92
Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P. Adult-onset Still’s disease. Autoimmun Rev. 2014; 13(7):708-22.
Buss SJ, Wolf D, Mereles D, et al. A rare case of reversible constrictive pericarditis with severe pericardial thickening in a patient with adult onset Stil’s disease. Int j Cardiol. 2010 Oct8;144(2):e23-5. doi: 10.1016/j.ijcard.2008.12.079. Epub 2009 Jan 30.
Bilska A, Willnska E, Sztumowicz M, et al. Reccurent effusive pericarditis in the course of adult-onset Still’s disease--case reports of two patients. Pneumonol Alergol Pol. 2011;79(3):215-21.
Dall’Ara F, Frassi M, Tincani A, et al. A retrospective study of patients with adult-onset Still’s disease: is pericarditis a possible predictor for biological disease-modifying antirheumatic drugs need? Clin Rheumatol. 2016;35(8)/2117-2123. doi:10.1007/s10067-015-3164- y. Epub 2016 Jan 12
Bodard Q., Langlois V., Guilpain P. Cardiac involvement in adult-onset Still’s disease: Manifestations, treatments and outcomes in a retrospective study of 28 patients. J Autoimmun. 2021;116:102541.
Gracia-Ramos A.E., Contreras-Ortiz J.A. Myocarditis in adult-onset Still’s disease: case-based review. Clin Rheumatol. 2020;39:933–947.
Gerfaud-Valentin M., Seve P., Iwaz J. Myocarditis in adult-onset still disease. Medicine (Baltimore) 2014;93:280–289.
Mavrogeni S.I., Kitas G.D., Dimitroulas T. Cardiovascular magnetic resonance in rheumatology: current status and recommendations for use. Int J Cardiol. 2016;217:135–148
Ertugrul BM, Uyar G, Ozturk B, Sakarya S. A rare presentation of endocarditis in adult onset Still’s disease in diagnosis of fever of unknown origin. J Rheumatol. 2012 Jan;39(1):198- 9. doi:10.38899/jrheum.110935.
Garcia-Porrua C, Gonzalez-Juanatey C, Gonzalez-Gay MA. Endocarditis in adult onset Still’s disease: a 12 month follow up. J Rheumatol. 2001 Sep;28(9):2141-2.
Narvaez J., Mora-Liminana M., Ros I. Pulmonary arterial hypertension in adult-onset Still's disease: a case series and systematic review of the literature. Semin Arthritis Rheum. 2019;49:162–170.
Narvaez J., Mora-Liminana M., Ros I. Pulmonary arterial hypertension in adult-onset Still's disease: a case series and systematic review of the literature. Semin Arthritis Rheum. 2019;49:162–170.
McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation. 2009 Apr 28;119(16):2250-94. doi:10.1161/CIRCULATIONAHA.109.192230. Epub 2009 Mar 30
Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2015; 46:903-975. doi:10.1183/13993003.01032-2015.
Lowther GH, Chertoff J, Cope J, et al. Pulmonary arterial hypertension and acute respiratory distress syndrome in a patient with adult-onset stills disease. Pulm Circ. 2017 OctDec;7(4):797-802. doi:10.1177/2045893217712710.7
