34. Felty Syndrome
Definition
Felty syndrome is a potentially serious condition associated with seropositive rheumatoid arthritis. It is characterized by rheumatoid arthritis along with splenomegaly and granulocytopenia.
Incidence
Felty syndrome is closely associated with rheumatoid arthritis, which affects about 1% of the general population. Of this 1%, Felty syndrome develops in about 1% to 3%. Therefore the estimated frequency is about 0.01% to 0.03% of the general population. Women are affected at a 3:1 ratio compared to men. It affects Caucasians more than any other race.
Etiology
The pathophysiologic origin of Felty syndrome is not fully understood. Granulocytes coated with immune complexes, diminished granulocyte growth factor levels, and circulating autoantibodies have been demonstrated, as well as linkages to a human leukocyte antigen genotype, suggesting a genetic contribution to development of the disorder.
Signs and Symptoms
• Episcleritis
• Extremity ischemia
• Hepatomegaly
• Joint deformities
• Lower extremity ulcers
• Lymphadenopathy
• Mononeuritis multiplex
• Peripheral neuropathy
• Periungual infarcts
• Pleuritis
• Portal hypertension
• Rheumatoid nodules
• Sjögren syndrome (see p. 316)
• Splenomegaly
• Synovitis
• Weight loss
Medical Management
Treatment of Felty syndrome relies heavily on treatment of the underlying rheumatoid arthritis. Traditionally this regimen has used gold salts as the pharmacologic mainstay. Response to these compounds is slow, however, and methotrexate has supplanted the gold salts in treating both rheumatoid arthritis and Felty syndrome. Cyclophosphamide may be useful in refractory cases, but the potential for leukopenia places distinct limitations on its use.
Etanercept and infliximab are being used to treat rheumatoid arthritis. Both act by blocking the effects of tumor necrosis factor–alpha. Neither has found a definite niche in the treatment of Felty syndrome.
Corticosteroids, specifically methylprednisolone, are frequently used to treat rheumatoid arthritis. The effectiveness is limited by time. The increased potential of infection has demoted these medications to second-line status in the treatment regimen.
Patients with intractable disease, who do not exhibit improvement via these other measures, may be recommended for splenectomy. Despite splenectomy, about 25% of patients experience recurrence of granulocytopenia.
Complications
• Bronchiolitis obliterans
• Congestive heart failure
• Gastrointestinal bleeding
• Immunosuppressive regimen toxicity
• Interstitial pneumonitis
• Life-threatening infection
• Myocarditis
• Pericarditis
• Pneumothorax
• Portal hypertension
• Pulmonary hypertension
• Ruptured spleen
Anesthesia Implications
Because of the close association of Felty syndrome with rheumatoid arthritis, the cervical spine, laryngeal structures, and temporomandibular joints are of particular interest to the anesthetist. Atlantoaxial subluxation is a potential complication during direct laryngoscopy. Symptoms of cervical lesions include occipital headache, nonspecific neck pain and/or stiffness, and stocking-glove distribution paresthesias.
Direct laryngoscopy may prove difficult secondary to severe limitation of the temporomandibular joints’ range of motion. Assessment of neck and jaw ranges of motion is essential in the preoperative evaluation of a patient with Felty syndrome.
The patient may have a restrictive lung disease, an obstructive disease, or a combination of both, which will be demonstrated by pulmonary function testing performed preoperatively. Pulmonary effects of rheumatoid arthritis include diffuse interstitial pneumonitis and fibrosis, pleurisy, pleural effusions, necrobiotic nodules, bronchiolitis obliterans, pulmonary arteritis, and apical fibrocavitary lesions. Because of these effects, the patient with Felty syndrome may develop pulmonary hypertension and pneumothorax. These pulmonary effects may also reduce the diffusing capacity of the patient. Restrictive effects can result in the loss of chest wall compliance.
