Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 6  |  Issue : 1  |  Page : 92-94  

A case report on Felty's syndrome


Department of Orthopaedics, Padmashree Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, India

Date of Web Publication14-Mar-2013

Correspondence Address:
Sahil Sanghi
Department of Orthopaedics, Padmashree Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.108662

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  Abstract 

A 35 years old female came to orthopedic OPD with multiple joint pain since 1 year with history of fever and morning stiffness without any history of trauma and previous infection. On examination there was small joint tenderness involving metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints with splenomegaly. Routine investigation revealed neutropenia and rheumatoid factor (RF) was positive. Patient was diagnosed to be suffering from Felty's syndrome. Treatment was initiated with methotrexate. Overall treatment was tolerated well, except for the development of mild fever, mild arthritis and transient thrombocytopenia. She is currently on methotrexate with reasonable control of her symptoms.

Keywords: Felty′s syndrome, MCP and PIP joint, neutropenia, RA factor, splenomegaly


How to cite this article:
Sanghi S, Aggarwal T, Salgia A, Biswas SK. A case report on Felty's syndrome. Med J DY Patil Univ 2013;6:92-4

How to cite this URL:
Sanghi S, Aggarwal T, Salgia A, Biswas SK. A case report on Felty's syndrome. Med J DY Patil Univ [serial online] 2013 [cited 2019 Oct 18];6:92-4. Available from: http://www.mjdrdypu.org/text.asp?2013/6/1/92/108662


  Introduction Top


Felty's Syndrome (FS) is a severe extra-articular feature of rheumatoid arthritis (RA). RA is a chronic inflammatory arthritis with significant extra-articular manifestations. Lifetime risk of FS for a RA patient is less than 1%. Over 95% of FS patients are positive for RF with high titers.


  Case Report Top


A 35 years old lady came with complaints of multiple joint pain including small joint of hand and morning stiffness since 1 year. She did not give history of trauma, previous infection or significant past medical history. Tenderness and swelling were present over wrists, MCP and PIP joints. She also had mild splenomegaly [Figure 1]. Subcutaneous nodules were present on forearm and small joints of hand [Figure 2].
Figure 1: Clinical photo showing extent of slenomegaly

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Figure 2: Clinical picture showing rheumatoid nodules with schwaan neck deformity of rt little finger

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  Routine Blood Investigation Showed Top


Hemoglobin was 8.6 g/dl.

White blood cell count was 1.2 × 10 9 /L

R.A. factor was positive.

C Reactive Protein (CRP) was positive.

Erythrocyte Sedimentation Rate (ESR) was 50 mm/hr.

Rest of the routine investigation including renal and liver function tests were within normal limits.

Xray of the wrists showed subluxation of MCP joint with arthritic changes more on right side with wrist joint changes [Figure 3].
Figure 3: X – Ray shows:- subluxation of mcp joint with arthritic changes more on rt side with wrist joint changes

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Ultrasonography USG abdomen/pelvis showed:-Showed splenomegaly.

Bone marrow biopsy: Showed normocellular and maturing hematopoiesis.

Clinical Impression: On basis of above finding she was diagnosed to be suffering from Felty's Syndrome (FS).


  Treatment Top


Treatment was initiated with a course of double drug disease-modifying anti-rheumatic drugs (DMARD) with Methotrexate 7.5 mg once weekly for 3 months and hydroxychloroquine (HCQ) 200 mg twice a day for 3 months. Supplementation with Folic acid 1 mg for rest of 6 days a week, Calcium 500 mg once daily and Vitamin D 250 I.U. once daily were also given to her. With this treatment she responded satisfactorily. She is being followed up every month.


  Discussion Top


FS is a severe extra-articular feature of RA. Lifetime risk of FS for a RA patient is less than 1%. [1] Over 95% of FS patients are positive for RF with high titers. [1],[2] FS usually develops after a long course of RA. [2] Arthritis almost always appears first and typically has been present for 10 years or more before neutropenia is recognized. [3] The articular disease in FS is usually severe in terms of both erosions and deformity. [2] In very rare cases, neutropenia appears before or with no arthritis and this patient is an example of the latter. [4],[5],[6],[7] Neutropenia and splenomegaly with elevated erythrocyte sedimentation rate, elevated C-reactive protein and anemia of chronic disease pointed toward connective tissue disorder. This case is an atypical presentation of FS because the lack of severe long lasting course of erosive RA. There is no specific diagnostic test for FS. It is a clinical diagnosis in RA with unexplained neutropenia and splenomegaly.

Treatment of neutropenia is mainly by disease-modifying anti-rheumatic drugs (DMARDs) including methotrexate, hydroxychloroquine, auranofin, penicillamine, gluco­corticoids, and G-CSF. The first choice for treating both neutropenia and arthritis is methotrexate which is safe, effective and well tolerated in these patients. [8] Recently, there has been an interest in the biologic agent rituximab in the treatment of FS but only a few cases has been reported. [9] Leflunomide, sulfasalazine and cyclophosphamide also have been reported but the experience is very limited. [10] The controlled studies of different treatment modalities are not available because of the rarity of this syndrome. Splenectomy produces a long-term hematologic response in 80% of patients but is usually reserved at the end of the treatment algorithm for treatment-resistant cases. [11]

G-CSF has no effect on the activity of RA but is effective and generally well-tolerated for the treatment of neutropenia due to FS. [12] Quick improvement in neutropenia has been reported with G-CSF. [13] G-CSF has been used as treatment for FS but with the known side effects of fever, thrombocytopenia and arthritis. The sustained granulopoietic response has been reported in some cases but the neutrophil count often declines when growth factor treatment is stopped but generally stabilized at a level that exceeds the pretreatment count. [12] Patients who tolerate G-CSF and have good hematologic responses may be candidates for prolonged therapy.

 
  References Top

1.Balint GP, Balint PV. Felty's syndrome. Best Pract Res Clin Rheumatol 2004;18:631-45.  Back to cited text no. 1
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2.Campion G, Maddison PJ, Goulding N, James I, Ahern MJ, Watt I, et al. The felty syndrome: A case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. Medicine (Baltimore) 1990;69:69-80.  Back to cited text no. 2
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3.Rosenstein ED, Kramer N. Felty's and pseudo-Felty's syndromes. Semin Arthritis Rheum 1991;21:129-42.  Back to cited text no. 3
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4.Heyn J. Non-articular Felty's syndrome. Scand J Rheumatol 1982;11:47-8.  Back to cited text no. 4
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5.Cornwell GG, Zacharski LR. Neutropenia, elevated rheumatoid factor, splenomegaly, and absence of rheumatoid arthritis. Ann Intern Med 1974;80:555-6.  Back to cited text no. 5
    
6.Bradley JD, Pinals RS. Felty's syndrome presenting without arthritis. Clin Exp Rheumatol 1983;1:257-9.  Back to cited text no. 6
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7.Armstrong RD, Fernandes L, Gibson T, Kauffmann EA. Felty's syndrome presenting without arthritis. Br Med J (Clin Res Ed) 1983;287:1620.  Back to cited text no. 7
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8.Wassenberg S, Herborn G, Rau R. Methotrexate treatment in Felty's syndrome. Br J Rheumatol 1998;37:908-11.  Back to cited text no. 8
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9.Chandra PA, Margulis Y, Schiff C. Rituximab is useful in the treatment of Felty's syndrome. Am J Ther 2008;15:321-2.  Back to cited text no. 9
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10.Wiesner KB, Shapiro RF, Bryan BL, Fuller C, Utsinger PD. Immunosuppressive therapy in Felty's syndrome. N Engl J Med 1977;296:1172.  Back to cited text no. 10
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11.Rashba EJ, Rowe JM, Packman CH. Treatment of the neutropenia of Felty syndrome. Blood Rev 1996;10:177-84.  Back to cited text no. 11
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12.Hellmich B, Schnabel A, Gross WL. Treatment of severe neutropenia due to Felty's syndrome or systemic lupus erythematosus with granulocyte colony-stimulating factor. Semin Arthritis Rheum 1999;29:82-99.  Back to cited text no. 12
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13.Moore DF Jr, Vadhan-Raj S. Sustained response in Felty's syndrome to prolonged administration of recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF). Am J Med 1995;98:591-4.  Back to cited text no. 13
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    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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Introduction
Case Report
Routine Blood In...
Treatment
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