Adult Onset Still’s Disease: Articular Manifestations in Twenty Cases

Abstract

The adult onset Still’s disease is a rare inflammatory pathology of unknown pathogeny. The clinical features are variable. The diagnosis is difficult since exclusion of infectious, systemic and tumoral pathologies should be done. The articular complications are frequent and can be revelatory of this pathology. The articular prognosis depends on the diagnosis delay and the treatment efficiency. Our study aims to analyze different aspects of articular manifestations complicating adult onset Still’s disease to define epidemiological, clinical and evolving characteristics of these complications. It was a cross-sectional study concerning 20 cases of adult onset Still’s disease diagnosed from 1990 to 2015 in the internal medicine A department of Charles Nicolle Hospital in Tunis, meeting Yamaguchi criteria. We identified clinical, radiological, evolving and therapeutic profile of the articular manifestations occurred in these patients. There were 13 women and 7 men. The average age was 25 years. The arthralgias were reported in all cases; while, the arthritis interested fifteen patients. A hand deformation was found in four patients. A wrist ankylosis was noted in one case and a flexion elbow in one patient. The standard articular radiographs were normal in twelve cases. The treatment associated essentially non-steroidal anti-inflammatory and/or corticosteroids and/or methotrexate. Concerning the evolving profile, the monocyclic form was present in 25% of the cases, the intermittent form in 45% and the chronic articular form in 30% of our patients. The adult onset Still’s disease is rare and heterogeneous. The articular disturbances are frequent and have various outcomes.

Share and Cite:

Mahfoudhi, M. , Gorsane, I. , Battikh, A. , Shimi, R. , Turki, S. , Hamida, F. and Abdallah, T. (2015) Adult Onset Still’s Disease: Articular Manifestations in Twenty Cases. Open Journal of Clinical Diagnostics, 5, 41-45. doi: 10.4236/ojcd.2015.52007.

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1] Yamaguchi, M., Ohta, A., Tsunematsu, T., Kasukawa, R., Mizushima, Y., Kashiwagi, H., et al. (1992) Preliminary Criteria for Classification of Adult Still’s Disease. The Journal of Rheumatology, 19, 424-430.
[2] Fautrel, B., Zing, E., Golmard, J.L., Le Moel, G., Bissery, A., Rioux, C., et al.. (2002) Proposal for a New Set of Classification Criteria for Adult-Onset Still Disease. Medicine (Baltimore), 81, 194-200.
http://dx.doi.org/10.1097/00005792-200205000-00003
[3] Magadur-Joly, G., Billaud, E., Barrier, J.H., Pennec, Y.L., Masson, C., Renou, P., et al. (1995) Epidemiology of Adult Still’s Disease: Estimate of the Incidence by a Retrospective Study in West France. Annals of Rheumatic Diseases, 54, 587-590.
http://dx.doi.org/10.1136/ard.54.7.587
[4] Efthimiou, P., Paik, P.K. and Bielory, L. (2006) Diagnosis and Management of Adult Onset Still’s Disease. Annals of Rheumatic Diseases, 65, 564-572.
http://dx.doi.org/10.1136/ard.2005.042143
[5] Meddeb, N., Amira, C., Elleuch, M., Cheour, E., Sahli, H., Hamza, S., et al. (2003) Articular Manifestations of Adult Still’s Disease. La Tunisie Médicale, 81, 245-249.
[6] Cheikhrouhou Abdelmoula, L., Tekaya, R., Ben Hadj Yahia, C., Chaabouni, L. and Zouari, R. (2007) Adult Onset Still’s Disease: About 11 Cases. La Tunisie Médicale, 85, 461-464.
[7] Ben Taarit, C., Turki, S. and Ben Maiz, H. (2002) Adult Still’s Disease: Study of a Series of 11 Cases. Journal des Maladies Vasculaires, 27, 31-35.
[8] Masson, C., Le Loët, X., Lioté, F., Renou, P., Dubost, J.J. and Boissier, M.C. (1995) Adult Still’s Disease: Part I. Manifestations and Complications in Sixty-Five Cases in France. Revue du Rhumatisme (English ed.), 62, 748-757.
[9] Riera, E., Olive, A., Narvaez, J., Holgado, S., Santo, P. and Mateo, L. (2011) Adult Onset Still’s Disease: Review of 41 Cases. Clinical and Experimental Rheumatology, 29, 331-336.
[10] Bywaters, EG. (1971) Still’s Disease in the Adult. Annals of Rheumatic Diseases, 30, 121-133.
http://dx.doi.org/10.1136/ard.2005.042143
[11] Ohta, A., Yamaguchi, M., Tsunematsu, T., Kasukawa, R., Mizushima, H., Kashiwagi, H., et al. (1990) Adult Still’s Disease: A Multicenter Survey of Japanese Patients. The Journal of Rheumatology, 17, 1058-1063.
[12] Evensen, K.J. and Nossent, H.C. (2006) Epidemiology and Outcome of Adult-Onset Still’s Disease in Northern Norway. Scandinavian Journal of Rheumatology, 35, 48-51.
http://dx.doi.org/10.1080/03009740510026616
[13] Fraisse, T.C., Degraeve, F., Rivière, S. and Le Quellec, A. (2006) Profils évolutifs et marqueurs pronostiques de la maladie de Still de l’adulte. Analyse d’une série monocentrique de 17 patients. La Revue de Médecine Interne, 27, 658-664.
http://dx.doi.org/10.1016/j.revmed.2006.05.003
[14] Uppal, S.S., Al-Mutairi, M., Hayat, S., Abraham, M. and Malaviya, A. (2007) Ten Years of Clinical Experience with Adult Onset Still’s Disease: Is the Outcome Improving? Clinical Rheumatology, 26, 1055-1060.
http://dx.doi.org/10.1007/s10067-006-0440-x
[15] Crispín, J.C., Martínez-Baños, D. and Alcocer-Varela, J. (2005) Adult-Onset Still Disease as the Cause of Fever of Unknown Origin. Medicine (Baltimore), 84, 331-337.
http://dx.doi.org/10.1097/01.md.0000188009.47085.76
[16] Nkoghe, D., Demonty, J., Leonard, P., Nnegue, S., Moutschen, M. and Kaye, O. (2002) Still’s Disease in the Adult. Revue Médicale de Liège, 57, 213-219.
[17] Singh, S., Samant, R. and Joshi, V.R. (2008) Adult Onset Still’s Disease: A Study of 14 Cases. Clinical Rheumatology, 27, 35-39.
http://dx.doi.org/10.1007/s10067-007-0648-4
[18] Van de Putte, L.B. and Wouters, J.M. (1991) Adult-Onset Still’s Disease. Baillière’s Clinical Rheumatology, 5, 263-275.
http://dx.doi.org/10.1016/S0950-3579(05)80283-3
[19] Poralla, T., Hütteroth, T., Mayet, W., Gutfreund, K., Hermann, E., Gerken, G., et al. (1988) Peritonitis and Massive Granulocytic Infiltration of the Spleen in Adult Still’s Disease. Zeitschrift für Rheumatologie, 47, 364-365.
[20] Bagnari, V., Colina, M., Ciancio, G., Govoni, M. and Trotta, F. (2010) Adult-Onset Still’s Disease. Rheumatology International, 30, 855-862.
http://dx.doi.org/10.1007/s00296-009-1291-y
[21] Pouchot, J., Sampalis, J.S., Beaudet, F., Carette, S., Décary, F., Salusinsky-Sternbach, M., et al. (1991) Adult Still’s Disease: Manifestations, Disease Course, and Outcome in 62 Patients. Medicine (Baltimore), 70, 118-136.
[22] Bagnari, V., Colina, M., Ciancio, G., Govoni, M. and Trotta, F. (2010) Adult-Onset Still’s Disease. Rheumatology International, 30, 855-862.
http://dx.doi.org/10.1007/s00296-009-1291-y
[23] Cush, J.J., Medsger Jr., T.A., Christy, W.C., Herbert, D.C. and Cooperstein, L.A. (1987) Adult Onset Still’s Disease. Clinical Course and Outcome. Arthritis & Rheumatology, 30, 186-194.
http://dx.doi.org/10.1002/art.1780300209
[24] Pouchot, J. and Vinceneux, P. (2004) Manifestations cliniques et biologiques de la maladie de Still de l’adulte. La Presse Médicale, 33, 1012-1018.
http://dx.doi.org/10.1016/S0755-4982(04)98830-6
[25] Fautrel, B., Borget, C., Rozenberg, S., Meyer, O., Le Loet, X., Masson, C., et al. (1999) Corticosteroid Sparing Effect of Low Dose Methotrexate Treatment in Adult Still’s Disease. The Journal of Rheumatology, 26, 373-378.
[26] Pouchot, J. and Arlet, J.B. (2012) Biological Treatment in Adult-Onset Still’s Disease. Best Practice & Research Clinical Rheumatology, 26, 477-487.
http://dx.doi.org/10.1016/j.berh.2012.07.002
[27] Debiais, S., Maillot, F., Luca, L., Buret, J., Fautrel, B. and Renard, J.P. (2008) Efficacy of Anakinra in a Case of Refractory Still Disease. Journal of Clinical Rheumatology, 14, 357-358.
http://dx.doi.org/10.1097/RHU.0b013e318188b8d8
[28] Kobayashi, M., Takahashi, Y., Yamashita, H., Kaneko, H. and Mimori, A. (2011) Benefit and a Possible Risk of Tocilizumab Therapy for Adult-Onset Still’s Disease Accompanied by Macrophage-Activation Syndrome. Modern Rheumatology, 21, 92-96.
http://dx.doi.org/10.3109/s10165-010-0348-9
[29] Kadavath, S. and Efthimiou, P. (2015) Adult-Onset Still’s Disease—Pathogenesis, Clinical Manifestations, and New Treatment Options. Annals of Medicine, 47, 6-14.
http://dx.doi.org/10.3109/07853890.2014.971052
[30] Jamilloux, Y., Gerfaud-Valentin, M., Henry, T. and Sève, P. (2014) Treatment of Adult-Onset Still’s Disease: A Review. Therapeutics and Clinical Risk Management, 11, 33-43.
[31] Cavalli, G., Franchini, S., Aiello, P., Guglielmi, B., Berti, A., Campochiaro, C., et al. (2015) Efficacy and Safety of Biological Agents in Adult-Onset Still’s Disease. Scandinavian Journal of Rheumatology, Epub ahead of print.
http://dx.doi.org/10.3109/03009742.2014.992949
[32] Medsger Jr., T.A. and Christy, W.C. (1976) Carpal Arthritis with Ankylosis in Late Onset Still’s Disease. Arthritis & Rheumatology, 19, 232-242.
http://dx.doi.org/10.1002/art.1780190216

Copyright © 2023 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.