Felty's syndrome without rheumatoid arthritis?

Area of Science:

• Rheumatology
• Hematology
• Immunology

Background:

• Felty's syndrome (FS) is defined by neutropenia and splenomegaly in patients with rheumatoid arthritis (RA) who are seropositive for rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibodies.
• Neutropenia in FS significantly increases susceptibility to infections, often appearing in patients with long-standing RA.

Observation:

• This case highlights a rare presentation of FS with severe neutropenia and mild splenomegaly in a patient with high RF and anti-CCP titers but without overt clinical RA symptoms.
• The patient's neutropenia persisted asymptomatically for four years before progressing to agranulocytosis.

Findings:

• Successful treatment with methotrexate and corticosteroids for the patient's neutropenia/agranulocytosis confirmed the autoimmune etiology of Felty's neutropenia.
• The response to standard anti-RA therapy underscores the autoimmune basis of neutropenia in this condition.

Implications:

• Recognizing Felty's syndrome, particularly non-articular forms, promptly is essential for appropriate management within the spectrum of autoimmune rheumatic diseases.
• This case emphasizes the importance of considering FS in patients with unexplained neutropenia, even with minimal or only laboratory evidence of RA.