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felty-syndrome

Felty Syndrome: Pathogenesis and clinical findings Authors: Anjali Arora Reviewers: Ben Campbell, Liam Martin*, Yan Yu* * MD at time of publication Longstanding chronic Rheumatoid Arthritis Rheumatoid Arthritis (RA): a disease of systemic autoimmunity – refer to Rheumatoid Arthritis slides for detailed pathogenesis Genetic susceptibility Presence of a specific type of Human Leukocyte Antigen (HLA-DR4), a surface protein on immune cells that is known to be associated with or worsen autoimmune activity Idiopathic autoimmunity Neutrophil activation, apoptosis and adherence to endothelial cells in the spleen Felty Syndrome An uncommon extraarticular manifestation of seropositive Rheumatoid Arthritis characterized by the triad of splenomegaly, neutropenia and arthritis Immunologic stress leads to proliferation of white pulp in spleen (responsible for initiating immune responses to foreign antigens) Splenomegaly ↑ RBC and platelet sequestration within the spleen leading to a quantitative shortage of these cells in circulation Enlarged spleen compresses stomach Loss of appetite Complex autoimmune phenomena lead to ↓ neutrophil production and ↑ removal from the circulating pool Neutropenia For a detailed mechanism, refer to Neutropenia slides ↓ Circulating neutrophils leads to ↑ susceptibility to infections Severe, untreated Rheumatoid Arthritis will lead to systemic inflammation See Rheumatoid Arthritis slides for detailed mechanism Chronic erosion of synovial membranes in joints commonly affecting the hands, wrists and knees Arthralgia (symmetric polyarthritis, usually with small joint distribution) Thrombocytopenia Refer to Thrombocytopenia slides for signs and symptoms Normocytic Anemia Refer to Normocytic Anemia slide for signs and symptoms Host becomes infected Fever Recurrent infections Legend: Pathophysiology Mechanism Sign/Symptom/Lab Finding Complications Published October 18, 2022 on www.thecalgaryguide.com