Felty Syndrome Clinical Presentation

Updated: Dec 13, 2019
  • Author: Richard M Keating, MD, FACR, FACP; Chief Editor: Herbert S Diamond, MD  more...
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Presentation

History

Many years of aggressive destructive rheumatoid arthritis (RA) precede the onset of Felty syndrome (FS). On occasion, RA and FS develop simultaneously. [8] The extra-articular manifestations of RA (eg, rheumatoid nodules, pleuropericarditis, vasculitis, peripheral neuropathy, episcleritis, other forms of eye involvement, Sjögren syndrome, adenopathy, skin ulcers) are more common in those patients who develop FS.

Patients with FS often report symptoms of mild inflammatory joint disease caused by synovitis; it is often the case that their burden of synovitis is less than that experienced previously. The history usually reveals a long preceding period of active and aggressive joint disease, which can be confirmed by means of physical examination and plain radiography. Some patients present with quiescent or so-called “burned-out” joint disease. A lack of synovitis or active joint disease should not dissuade the clinician from considering the diagnosis of FS in an RA patient.

Patients with FS commonly present with bacterial infections of the skin and respiratory tract. An aggressive level of immunosuppression directed at the underlying RA may contribute to this susceptibility to infection.

Patients may present with left upper quadrant pain, initiated by splenic infarcts or capsular distension of the spleen.

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Physical Examination

Physical findings in FS include the following:

  • Splenomegaly, possibly nonpalpable

  • Hepatomegaly, usually mild

  • Lymphadenopathy

  • Weight loss

  • Rheumatoid nodules

  • Sjögren syndrome

  • Articular findings of long-standing RA – Joint deformities typical of RA, as well as synovitis (joint swelling and tenderness), which may be mild at presentation

  • Small-vessel inflammation (vasculitis) – Lower-extremity ulcers, palpable purpura and brownish pigmentary changes of the lower extremities, and periungual infarcts

  • Signs of systemic vasculitis – Mononeuritis multiplex and extremity ischemia

  • Other findings – Pleuritis, peripheral neuropathy, episcleritis, and signs of portal hypertension

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Complications

Complications of FS include the following:

  • Life-threatening infection

  • Toxicity due to immunosuppressive regimens

  • Portal hypertension and gastrointestinal bleeding due to nodular regenerative hyperplasia of the liver

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