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Felty Syndrome

 

Felty syndrome: Triad of

Rheumatoid arthritis (RA)

Splenomegaly

Neutropenia

 

IMAGING

Usually severely deforming RA

Erosions

Cartilage destruction

Joint deformities

 

May have other extraarticular manifestations of RA

Rheumatoid nodules

Vasculitis

Sjögren syndrome

Pericarditis

 

CLINICAL ISSUES

Rare process

Usually Caucasian female

High titer rheumatoid factor (RF)

Often have HLA-DR4 and other alleles associated with disease severity in RA

Etiology unknown

Neutropenia may result from splenic sequestration of granulocytes

Treatment parallels that of RA

Disease-modifying drug therapy; rituximab (monoclonal anti CD20 antibody) reported effective

Arthroplasty, arthrodesis as needed to regain function

Consider splenectomy if

Splenomegaly is massive

Patient suffers recurrent infections

Associated large granular lymphocytic leukemia in 40% of cases

Most cases of neutropenia in RA are acquired, not Felty syndrome

Related to medications used for RA

 






Anteroposterior radiograph shows splenomegaly , along with severe erosive disease of the hips, resulting in protrusio . This is a typical case of Felty syndrome; the patient also had neutropenia. In this rare syndrome, the arthritic process is usually severe, as in this case.

 

 





Anteroposterior radiograph shows severe splenomegaly  as outlined by the bowel gas in the splenic flexure. No other abnormality is present, and there is a wide differential based on this image alone.

 





AP radiograph of the forefoot in the same patient shows joint space narrowing and erosive change of the 5th metatarsal head .

 



AP radiograph of the contralateral foot shows a symmetric pattern of cartilage narrowing and erosive change at the 5th metatarsophalangeal joint . The patient proved to have rheumatoid arthritis and neutropenia, as well as the noted splenomegaly, completing the triad of Felty syndrome. It is unusual for the RA to appear so mild in a patient with Felty syndrome.


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