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Extended matching item (EMI)
Published in Tristan Barrett, Nadeem Shaida, Ashley Shaw, Adrian K. Dixon, Radiology for Undergraduate Finals and Foundation Years, 2018
Tristan Barrett, Nadeem Shaida, Ashley Shaw, Adrian K. Dixon
Still’s disease is characterised by rheumatoid arthritis, a salmon-pink rash, pyrexia, hepatosplenomegaly and lymphadenopathy; rheumatoid factor and anti-nuclear antibodies are usually negative. Felty’s syndrome is defined by the presence of rheumatoid arthritis, splenomegaly, and neutropaenia; it affects < 1% of RhA patients.
Station 1: Abdominal
Published in Saira Ghafur, Parminder K Judge, Richard Kitchen, Samuel Blows, Fiona Moss, The MRCP PACES Handbook, 2017
Saira Ghafur, Parminder K Judge, Richard Kitchen, Samuel Blows, Fiona Moss
Look for other signs leading to the underlying cause of the splenomegaly: Felty’s syndrome Rheumatoid hand signsPortal hypertension Signs of chronic liver diseaseInfective endocarditis Murmur, splinter haemorrhagesCML/CLL/lymphoma Lymphadenopathy (CLL/lymphoma), pallor
Arthritis
Published in Harry Griffiths, Musculoskeletal Radiology, 2008
Felty’s syndrome refers to a rare form of juvenile chronic arthritis (usually rheumatoid factor–positive), which is associated with splenomegaly and neutropenia. The systemic manifestations of the disease are serious, including recurrent infections, as well as a severe, destructive arthritis.
Successful treatment of refractory neutropenia in Felty’s syndrome with rituximab
Published in Scandinavian Journal of Rheumatology, 2018
Felty’s syndrome (FS) is characterized by a triad of severe, long-standing, and seropositive rheumatoid arthritis (RA), neutropenia, and splenomegaly, as first described in 1924 by an American physician, Augustus Roi Felty (1). It is a very rare systemic complication in RA, especially in Han Chinese patients, with an occurrence of less than 0.1% (2, 3). Owing to an increased risk of serious infection, the overall mortality is rather high in this disorder (4). Conventional therapeutic modalities for managing the neutropenic status include glucocorticoids, methotrexate, granulocyte colony-stimulating factor (G-CSF), and splenectomy; however, the results of these treatments are unpredictable as there have been no controlled trials in this rare disorder (3, 4). In southern Taiwan, with a Han Chinese-dominant population, there is an increasing trend towards the use of biologicals in rheumatological practice (5). A FS patient is reported here with long-lasting RA activity, severe neutropenia, and massive splenomegaly. Regular rituximab (RTX) infusion prescribed every 6 months resulted in normalization of the absolute neutrophil count (ANC) and significantly reduced arthritis activity.
Successful abatacept treatment for Felty’s syndrome in a patient with rheumatoid arthritis
Published in Modern Rheumatology Case Reports, 2020
Felty’s syndrome is extra-articular manifestation of rheumatoid arthritis (RA) characterised by neutropenia and splenomegaly [1]. It affects <1% of patients with RA [1]. In patients with Felty’s syndrome, severe neutropenia is associated with risk of serious infection. Conventional treatment of neutropenia in Felty’s syndrome consists of corticosteroids, granulocyte colony-stimulating factor, and conventional disease modifying anti-rheumatic drugs (DMARDs). In biologic DMARDs, previous reports reviewed that rituximab treatments result in increased neutrophil, but not tumour necrosis factor inhibitors [2–5]. However, there is only one case report that treated for Felty’s syndrome with ABT [6].