Study: Felty’s Syndrome Presents Differently in Pediatric Patients

According to a study published in BMC Pediatrics, the rare disease known as Felty’s syndrome is primarily known to affects adults between the ages of 50 and 70, but on very rare occasions, it can impact children as well. This case study reveals that this disease presents with different symptoms in pediatric patients in comparison to adults. The study examines the case of a 14 year old girl affected by Felty’s syndrome.

About Felty’s Syndrome

Felty’s syndrome is a rare autoimmune disease usually characterized by three distinct manifestations: neutropenia (low neutrophil cell count), rheumatoid arthritis, and enlarged spleen. The disease is more common in women and people of European descent. It generally affects people living with long-term rheumatoid arthritis and can be described as a systemic progression of the disease. Symptoms include joint stiffness, pain and swelling; typically the hands, feet, and arms are affected. Other symptoms include enlarged spleen, neutropenia, fever, weight loss, sores on the legs, fatigue, skin discoloration, anemia, enlarged liver, enlarge lymph nodes, and infections. Overall, the best way to treat Felty’s syndrome is to bring the underlying arthritis under control; rituximab can be an effective choice for this task. Outcomes vary depending on the patient’s overall health, presence of comorbidities, and severity of symptoms. To learn more about Felty’s syndrome, click here.

Case Study

In a very small number of cases, Felty’s syndrome has appeared in young people with chronic juvenile idiopathic arthritis (JIA). The case patient had a seven year history of the disease with multiple joint involvement. As her disease began to progress, she began to present with a variety of systemic symptoms. These include cholestasis (lack of bile flow), enlarged spleen, enlarged liver, and thrombocytopenia (abnormally low platelet count in the blood). She also occasionally presented with neutropenia as well.

In an attempt to resolve these symptoms, a variety of therapies were administered. These included sulfasalazine, hydroxychloroquine, methotrexate, prednisone, and rituximab. The only therapy that the patient responded to was rituximab. Ultimately the researchers concluded that pediatric patients are more likely to present with symptoms of enlarged spleen, thrombocytopenia, and juvenile idiopathic arthritis, with neutropenia only appearing intermittently. However, cases where no neutropenia appears are probably very uncommon.

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