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FELTY’S SYNDROME

Men's Health, Women's Health / By

FELTY’S SYNDROME

Felty’s syndrome is a rare, potentially serious autoimmune disease defined by the presence of rheumatoid arthritis, enlargement of the spleen (splenomegaly), and an abnormally low white blood cell count (neutropenia).

  • People who suffer from felty’s syndrome are increasingly susceptible to infections than an average person because they have a low white blood cell count.
  • This condition is more common in those aged 50-70 years, especially those who have had rheumatoid arthritis for 10 years or longer. It is seen in 1-3% of cases of rheumatoid arthritis.
  • It is also three times more prevalent in women than in men, and rarely affects children.
  • It is also common among Caucasians than those of African descent.

CAUSES

The exact cause of Felty’s syndrome is unknown, but it has been found to be more common in people with chronic rheumatoid arthritis. Some experts believe that it may sometimes be inherited in an autosomal dominant manner. Other proposed causes and risk factors include:

  • Aggressive and erosive inflammation of the tissue lining the joints
  • Auto-immune disorder, where the immune system attacks the white blood cells mistakenly
  • HLA-DR4 genotype. This genotype is a marker for more aggressive rheumatoid arthritis.

SYMPTOMS

Symptoms of Felty’s syndrome are similar to those of rheumatoid arthritis and may vary from person to person. Patients suffer from:

  • Painful, stiff, and swollen joints most commonly in the joints of the feet, hands, and arm.
  • Abnormally enlarged spleen
  • Abnormally low levels of certain white blood cells
  • Fever
  • Weight loss
  • Fatigue
  • Discoloration of the skin (abnormal brown pigmentation), particularly of the leg
  • Ulcers on the lower leg
  • Anemia
  • Inflammation of the blood vessels
  • Abnormally large liver
  • Swollen lymph nodes
  • Recurrent sore throat
  • Nasal inflammation
  • Bone marrow failure
  • Bacterial infection of the skin
  • Infection in the blood stream
  • Frequent urinary tract infection

DIAGNOSIS AND TREATMENT

Diagnosis is based on the presence of rheumatoid arthritis, splenomegaly, and neutropenia.

To make a diagnosis, the doctor will review your medical history and carry out a physical exam.  During the physical exam, the doctor will palpate for swollen spleen, observe joints that show signs of rheumatoid arthritis, palpate for swollen liver and lymph node.

A complete blood count (CDC) can be done to show anemia, a low number of white blood cells called neutrophils.

An imaging test such as MRI, CT scan, or ultrasound to help diagnose enlarged spleen

Bone marrow biopsy to help differentiate felty’s syndrome from hematological malignancies such as non-Hodgkin’s lymphoma.

TREATMENT

The goal of the treatment option is to control and address presenting symptoms

Treatment options include:

  • Immunosuppressive therapy for rheumatoid arthritis
  • Physical therapy
  • Methotrexate to improve the low white blood cell count
  • Granulocyte colony-stimulating factor (G-CSF) to help increase the number of white blood cells and help fight infection.
  • Surgery to remove your spleen if your condition is severe and other treatments don’t work