Felty’s syndrome diagnosis issue and its formulation problem
Yu.V. Muraviev
V.A. Nasonova Research Institute of Rheumatology, Moscow
The article is devoted to a controversial issue caused by the lack of consensus in the medical literature regarding the Felty’s syndrome (FS) diagnosis formulation, which actually is rheumatoid arthritis (RA) with extra-articular (hematological) manifestations. In most FS cases, there is a typical for RA damage of predominantly hands and feet small joints of varying severity. The following extra-articular RA manifestations are the most common: subcutaneous nodules, polyneuropathy, generalized lymphadenopathy, hepatomegaly, polyserositis, myocarditis, Sjogren’s syndrome signs. An increased incidence of infectious diseases is explained by a decrease in the leukocytes main functions (phagocytosis, chemotaxis, bactericidal activity). The spleen size increases significantly, with an average of 4 times its normal weight with this syndrome. The diagnosis is made on the basis of the neutropenia and splenomegaly manifestations in patients with RA. The article deals with the unification question of the diagnosis formulation, which would allow not only to improve statistical accounting but also to monitor this pathology.
Keywords: Felty’s syndrome, rheumatoid arthritis, extra-articular rheumatoid arthritis manifestations, neutropenia, leukopenia, splenomegaly.
For citation: Muraviev Yu.V. Felty’s syndrome diagnosis issue and its formulation problem. RMJ. Medical Review. 2019;4(I):2–3.
References
1. Felty A.R. Chronic arthritis in the adult associated with splenomegaly and leucopenia. Bulletin of the Johns Hopkins Hospital. 1924;35:16–20.
2. Hanrahan E.M., Miller S.R. Effect of splenectomy in Felty’s syndrome. JAMA. 1932;99:1247–1249.
3. Ruderman M., Miller L.M., Pinals R.S. Clinical and serological observations on 27 patients with Felty’s syndrome. Arthritis Rheum. 1968;11(3):377–384.
4. Barnes C.C., Turnbull A.L., Vernon-Roberts B. Felty’s syndrome. A clinical and pathological survey of 21 patients and their response to treatment. Ann Rheum Dis. 1971;30:359–374.
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11. Sienknecht C.W., Urowitz M.B., Pruzanski W., Stein H.B. Felty’s syndrome. Clinical and serological analysis of 34 cases. Annals of the Rheumatic Diseases. 1977;36:500–507.
12. Campion G., Maddison P.J., Goulding N. et al. The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. Medicine. 1990;69:69–80.
13. Denko C.W., Zumpft C.W. Chronic arthritis with splenomegaly and leukopenia. Arthritis Rheum. 1962;5:478–491.
14. Rosenstein E.D., Kramer N. Felty’s and pseudo-Felty’s syndromes.Semin Arthritis Rheum. 1991;21(3):129–142.
15. Breedveld F.C., Fibbe W.E., Cats A. Neutropenia and infections in Felty’s syndrome. British Journal of Rheumatology. 1988;27:191–197.
16. Cornwell G.G., Zacharski L.R. Neutropenia, elevated rheumatoid factor, splenomegaly, and absence of rheumatoid arthritis. Annals of Internal Medicine. 1974;80:555–556.
17. Bradley J.D., Pinals R.S. Felty’s syndrome presenting without arthritis. Clin Exp Rheumatol. 1983;1:257–259.
18. Armstrong R.D., Fernandes L., Gibson T. et al. Felty’s syndrome presenting without arthritis. Br Med J. 1983;287:1620.
19. Moore R.A., Brunner C.M., Sandusky W.R., Leavell B.S. Felty’s syndrome: long-term follow-up after splenectomy. Ann Intern Med. 1971;75(3):381–385.
20. Bucknall R.C., Davis P., Bacon P.A., Jones J.V. Neutropenia in rheumatoid arthritis: studies on possible contributing factors. Annals of the Rheumatic Diseases. 1982;41:242–247.
21. Thorne C., Urowitz M.B. Long-term outcome in Felty’s syndrome. Annals of the Rheumatic Diseases. 1982;41:486–489.
22. Муравьев Ю.В. Внесуставные проявления ревматоидного артрита. Научно-практическая ревматология. 2018;56(3):356–362. Muravyev Y.V. Extra-articular manifestation of rheumatoid arthritis. Rheumatology Science and Practice. 2018;56(3):356–362 (in Russ.).
23. Richman N.C., Yazdany J., Graf J. et al. Extraarticular manifestations of rheumatoid arthritis in a multiethnic cohort of predominantly Hispanic and Asian patients. Medicine (Baltimore). 2013;92(2):92–97.
24. Das S., Padhan P. An Overview of the Extraarticular Involvement in Rheumatoid Arthritis and its Management. J Pharmacol Pharmacother. 2017;8(3):81–86.
2. Hanrahan E.M., Miller S.R. Effect of splenectomy in Felty’s syndrome. JAMA. 1932;99:1247–1249.
3. Ruderman M., Miller L.M., Pinals R.S. Clinical and serological observations on 27 patients with Felty’s syndrome. Arthritis Rheum. 1968;11(3):377–384.
4. Barnes C.C., Turnbull A.L., Vernon-Roberts B. Felty’s syndrome. A clinical and pathological survey of 21 patients and their response to treatment. Ann Rheum Dis. 1971;30:359–374.
5. Насонова В.А. Фелти синдром. Большая медицинская энциклопедия: в 30 т. Гл. ред. Б.В. Петровский. 3-е изд. М.: Советская энциклопедия. Т. 26. 1985:243–244.
6. Насонова В.А., Уметова М.Д., Сперанский А.И. Клинико-иммунологические особенности синдрома Фелти. Терапевтический архив.1980;4:105–109.
7. Международная статистическая классификация болезней и проблем, связанных со здоровьем. 10-й пересмотр. Т. 1 (ч. 1). Всемирная организация здравоохранения. Женева; 1995.
8. Ревматология: Клинические рекомендации. Под ред. акад. РАМН Е.Л. Насонова.2-е изд., испр. и доп. М.: ГЭОТАР-Медиа; 2010:752.
9. Российские клинические рекомендации. Ревматология. Под ред. Е.Л. Насонова. М.: ГЭОТАР-Медиа; 2017:464.
10. Goldberg J., Pinals R.S. Felty syndrome. Seminars in Arthritis and Rheumatism. 1980;10:52–65.
11. Sienknecht C.W., Urowitz M.B., Pruzanski W., Stein H.B. Felty’s syndrome. Clinical and serological analysis of 34 cases. Annals of the Rheumatic Diseases. 1977;36:500–507.
12. Campion G., Maddison P.J., Goulding N. et al. The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. Medicine. 1990;69:69–80.
13. Denko C.W., Zumpft C.W. Chronic arthritis with splenomegaly and leukopenia. Arthritis Rheum. 1962;5:478–491.
14. Rosenstein E.D., Kramer N. Felty’s and pseudo-Felty’s syndromes.Semin Arthritis Rheum. 1991;21(3):129–142.
15. Breedveld F.C., Fibbe W.E., Cats A. Neutropenia and infections in Felty’s syndrome. British Journal of Rheumatology. 1988;27:191–197.
16. Cornwell G.G., Zacharski L.R. Neutropenia, elevated rheumatoid factor, splenomegaly, and absence of rheumatoid arthritis. Annals of Internal Medicine. 1974;80:555–556.
17. Bradley J.D., Pinals R.S. Felty’s syndrome presenting without arthritis. Clin Exp Rheumatol. 1983;1:257–259.
18. Armstrong R.D., Fernandes L., Gibson T. et al. Felty’s syndrome presenting without arthritis. Br Med J. 1983;287:1620.
19. Moore R.A., Brunner C.M., Sandusky W.R., Leavell B.S. Felty’s syndrome: long-term follow-up after splenectomy. Ann Intern Med. 1971;75(3):381–385.
20. Bucknall R.C., Davis P., Bacon P.A., Jones J.V. Neutropenia in rheumatoid arthritis: studies on possible contributing factors. Annals of the Rheumatic Diseases. 1982;41:242–247.
21. Thorne C., Urowitz M.B. Long-term outcome in Felty’s syndrome. Annals of the Rheumatic Diseases. 1982;41:486–489.
22. Муравьев Ю.В. Внесуставные проявления ревматоидного артрита. Научно-практическая ревматология. 2018;56(3):356–362. Muravyev Y.V. Extra-articular manifestation of rheumatoid arthritis. Rheumatology Science and Practice. 2018;56(3):356–362 (in Russ.).
23. Richman N.C., Yazdany J., Graf J. et al. Extraarticular manifestations of rheumatoid arthritis in a multiethnic cohort of predominantly Hispanic and Asian patients. Medicine (Baltimore). 2013;92(2):92–97.
24. Das S., Padhan P. An Overview of the Extraarticular Involvement in Rheumatoid Arthritis and its Management. J Pharmacol Pharmacother. 2017;8(3):81–86.
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