Primary Sjogren’s Syndrome (pSS) is a classic, immunemediated, condition of unknown aetiology characterized by focal lymphocytic infiltration of exocrine glands [1]. Patients characteristically complain of drying of the eyes and mucosal surfaces along with fatigue and arthralgia. There is an association with autoimmune thyroid disease, coeliac disease and primary biliary cirrhosis. Systemic features include inflammatory arthritis, scLE, immune thrombocytopenia (ITP), vasculitis with purpura, salivary gland inflammation, neuropathies, interstitial lung disease (ILD) and a 510% lifetime risk of B cell lymphoma [2, 3]. This guideline reviews the treatment of the glandular and systemic features of pSS The management of the glandular features includes conserving, replacing and stimulating secretions. Systemic features may require system-specific therapy and immunomodulatory treatment. Holistic management is important and many patients benefit from non-pharmacological therapies and general support.
The British Society for Rheumatology guideline for the management of adults with primary Sjögren's Syndrome / Price, Elizabeth J.; Rauz, Saaeha; Tappuni, Anwar R.; Sutcliffe, Nurhan; Hackett, Katie L.; Barone, Francesca; Granata, Guido; Ng, Wan-Fai; Fisher, Benjamin A.; Bombardieri, Michele; Astorri, Elisa; Empson, Ben; Larkin, Genevieve; Crampton, Bridget; Bowman, Simon J.. - In: RHEUMATOLOGY. - ISSN 1462-0324. - 56:10(2017), pp. e24-e48. [10.1093/rheumatology/kex166]
The British Society for Rheumatology guideline for the management of adults with primary Sjögren's Syndrome
Granata, Guido;Astorri, Elisa;
2017
Abstract
Primary Sjogren’s Syndrome (pSS) is a classic, immunemediated, condition of unknown aetiology characterized by focal lymphocytic infiltration of exocrine glands [1]. Patients characteristically complain of drying of the eyes and mucosal surfaces along with fatigue and arthralgia. There is an association with autoimmune thyroid disease, coeliac disease and primary biliary cirrhosis. Systemic features include inflammatory arthritis, scLE, immune thrombocytopenia (ITP), vasculitis with purpura, salivary gland inflammation, neuropathies, interstitial lung disease (ILD) and a 510% lifetime risk of B cell lymphoma [2, 3]. This guideline reviews the treatment of the glandular and systemic features of pSS The management of the glandular features includes conserving, replacing and stimulating secretions. Systemic features may require system-specific therapy and immunomodulatory treatment. Holistic management is important and many patients benefit from non-pharmacological therapies and general support.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
