{"id":8432,"date":"2021-11-25T19:22:33","date_gmt":"2021-11-25T17:22:33","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/rheumatology\/special-forms-of-rheumatoid-arthritis-felty-caplan-syndrome\/"},"modified":"2022-04-21T20:02:40","modified_gmt":"2022-04-21T18:02:40","slug":"special-forms-of-rheumatoid-arthritis-felty-caplan-syndrome","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/rheumatology\/special-forms-of-rheumatoid-arthritis-felty-caplan-syndrome\/","title":{"rendered":"Special forms of rheumatoid arthritis \u2013 Felty, Caplan syndrome"},"content":{"rendered":"\n

Felty’s syndrome<\/h3>\n<\/span>\n\n\n

Felty’s syndrome (FS) is a uncommon and severe form of RA in which innate immunity is severely reduced, leading to recurrent infections.<\/p>\n\n\n\n

  • FS is characterized by a triad of neutropenia<\/strong><\/strong>, rheumatoid arthritis<\/strong>, and splenomegaly<\/strong>.<\/li>
  • Neutropenia is the most common and important feature (not all patients have splenomegaly or active RF).<\/li>
  • Etiology.<\/strong> The neutropenia is thought to be due to bone marrow failure, excessive NETosis, and their increased destruction in the spleen.
    • Genetics<\/strong>. Associated with HLA-DR4.<\/li><\/ul><\/li>
    • Clinical presentation<\/strong>. Patients with FS may be asymptomatic or suffer from recurrent infections (most often of the mouth and respiratory tract), fever, lymphadenopathy, joint pain and destruction (RA), anemia, and liver failure.
      • Patients with FS have 2-fold risk for malignancies.<\/li><\/ul><\/li>
      • Diagnosis<\/strong>
        • TBC (neutropenia)<\/li>
        • Serology (antihistone antibodies)<\/li>
        • Lymph node, and spleen biopsy (for ruling out lymphoma)<\/li>
        • Bone-marrow biopsy (BM failure)<\/li><\/ul><\/li>
        • Treatment<\/strong>
          • Treatment of RA (NSAIDs, local corticoids, DMARDs)<\/li>
          • Broad-spectrum antibiotics<\/li>
          • Splenectomy<\/li>
          • Low-dose G-CSF (granulocyte colony-stimulating factor) is an experimental treatment for neutropenia.<\/li><\/ul><\/li><\/ul>\n\n\n<\/span>\n

            Caplan syndrome<\/h3>\n<\/span>\n\n\n

            Caplan syndrome, also known as rheumatoid pneumoconiosis<\/strong>, is the combination of rheumatoid arthritis and pneumoconiosis. It occurs in RA patients who are chronically exposed to mineral dust such as coal, asbestos, silica.<\/p>\n\n\n\n

            • Etiology<\/strong>. It is thought that patients with RA show an increased immune reaction to foreign material such as mineral dusts in their airways. The inhaled dusts cause an inflammatory reaction and autoimmunity.<\/li>
            • Clinical presentation<\/strong>. Caplan syndrome presents with dyspnea appearing together with arthritis.
              • The condition may progress to pulmonary fibrosis.<\/li><\/ul><\/li>
              • Diagnosis<\/strong>
                • Clinically (dyspnea + RA)<\/li>
                • X-ray shows small nodules resembling tuberculosis; tuberculin test for the exclusion of TB<\/li>
                • Serology (RF, ACPA)<\/li>
                • Lung function tests<\/li>
                • Lung biopsy<\/li><\/ul><\/li>
                • Management.<\/strong> Avoidance of the dust exposure and steroids (after the exclusion of TB), and treatment of the RA.<\/li>
                • Asbestosis, silicosis, and tuberculosis are a differential diagnosis and must be excluded.<\/li><\/ul>\n<\/span>

                  Felty’s syndrome<\/h3>

                  Caplan syndrome<\/h3><\/div>","protected":false},"excerpt":{"rendered":"

                  Felty’s syndrome Felty’s syndrome (FS) is a uncommon and severe form of RA in which innate immunity is severely reduced, leading to recurrent infections. FS is characterized by a triad of neutropenia, rheumatoid arthritis, and splenomegaly. Neutropenia is the most common and important feature (not all patients have splenomegaly or active RF). Etiology. The neutropenia […]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":8424,"menu_order":3,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-8432","page","type-page","status-publish","hentry"],"yoast_head":"\nSpecial forms of rheumatoid arthritis \u2013 Felty, Caplan syndrome – Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/rheumatology\/special-forms-of-rheumatoid-arthritis-felty-caplan-syndrome\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"2 minutes\" \/>\n<script type=\"application\/ld+json\" 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