The clinical spectrum of primary Sjögren’s syndrome: beyond exocrine glands

Abstract

SUMMARYAlthough primary Sjögren's syndrome (pSS) is a mild indolent chronic disease mainly characterized by mucosal dryness in the majority of cases, a consistent subgroup of patients display extra-glandular manifestations. Virtually any organs and systems can be affected, leading to a more serious disease prognosis. Therefore, the prompt identification of patients at higher risk of extra-glandular manifestations is necessary to start a thorough follow up and an aggressive treatment. The aim of this review article is to provide an overview of epidemiological, clinical and serological features of extra-glandular manifestations in pSS as well as current knowledge about putative biomarkers useful in clinical practice. Key words:Sjögren's syndrome; Extra-glandular manifestations; Cardiovascular risk. Reumatismo, 2017; 69 (3): 93-100 n INTRODUCION P rimary Sjögren's syndrome (pSS) is a complex and heterogeneous chronic autoimmune disease primarily characterized by a focal lymphocytic chronic inflammation of exocrine glands leading to progressive loss of secretory function (1). Therefore, the specific case-history of pSS is dominated by signs and symptoms of mucosal dryness. However, although salivary and lachrymal glands represent the main target of pSS, several other organs and systems may be affected during the disease course in at least one third of patients, resulting in a plethora of systemic clinical manifestations, serological abnormalities and complications (2). The most severe complication of pSS is non-Hodgkin lymphoma occurring in about 5% of patients (3) and dramatically worsening disease prognosis. In this regard, the prompt identification of pSS patients with higher risk of developing extraglandular manifestations is mandatory for correct management. Low C3 and C4 levels, cryoglobulins, monoclonal component, anti-Ro/ SSA, anti-La/SSB, rheumatoid factor and hypergammaglobulinaemia represent poor prognostic serological factors in pSS associated with lymphoma and severe extraglandular features (2). Furthermore, histological features such as the presence of germinal centre-like structures in the minor salivary glands have been associated with higher risk of developing lymphoma (4). The purpose of this review article is to discuss current knowledge of extra-glandular manifestations in pSS not secondary to mucosal dryness, excluding pathogenesis and clinical manifestations of lymphoproliferative disorders. n MUSCULOSKELETAL INVOLVEMENTMusculoskeletal manifestations such as myalgia, arthralgia and morning stiffness are present in as many as 90% of patients, while clinically evident arthritis is found in up to 17%. In a recent study conducted on 17 female patients with pSS and 18 patients with secondary SS (sSS), ultrasonography (US) was used to confirm the non-erosive nature of joint involvement in pSS and to discriminate between pSS and RA-associated SS. Synovitis was commonly detected in pSS, mainly in metacarpophalangeal joints (MCP), wrists, and knees. Bone erosions also may occur. Power Doppler signal ...

Sjögren’s syndrome: a systemic autoimmune disease

“…Arthritis is predominantly symmetrical, generally mono-or oligoarticular with the involvement of proximal interphalangeal, metacarpophalangeal joints and wrists [91]. The large majority of SS-related arthritis is nonerosive, while synovitis and bone erosions are characteristic features of secondary SS associated with RA [90,91]. In SS patients, true myositis is rare, while myalgias and/or muscle weakness are relatively frequent and can be secondary to fibromyalgia or hypokalemia, respectively [92,93].…”

Hypokalemic Paralysis Secondary to Renal Tubular Acidosis Revealing Underlying Sjogren's Syndrome

“…Sjogren's syndrome is a systemic autoimmune disease characterized by cell-mediated autoimmunity against exocrine glands. Extraglandular manifestations of the disease arise from a similar pathogenetic mechanism affecting the kidneys, liver, lungs, pancreas, and the nervous system [ 10 ]. Renal involvement in Sjogren's syndrome is rare, affecting <10% of patients [ 11 ].…”

Sjögren Sendromunda Nörolojik Tutulum: Olgular Temelinde Gözden Geçirme

“…Pozitif tükürük bezi biyopsisi (Evre 3-4 inflamasyon) ve klinik bulgular SS tanısı koymada en etkin yöntemler olarak belirtilmektedir. [1][2][3][4][5] Escudero ve ark. SS'de santral sinir sistemi bulguları olarak kronik baş ağrısı %52, temporomandibuler eklem ağrısını ise %80 oranında bildirilmişlerdir.…”

“…Ekstraglandüler bulguların nedeni yol açtığı lenfositik vaskülittir. [1,2] Hastalığın tanısı ve sınıflandırılmasına yönelik kabul gören son ölçütler; 2016'da Amerikan Romatoloji Birliği ve Avrupa Romatoloji Birliği (ACR/EULAR) tarafında belirlenen ve bulguların ağırlık derecelerinin hesaplandığı sette yer almaktadır. Buna göre ağırlık skoru ≥4 olan olgularda SS tanısı koyulması önerilmiştir.…”

“…Birincisi, sinir sisteminin mononükleer hücrelerle infiltrasyonu, ikincisi ise anti-Ro ve antinöronal antikorlarla ilişikili vaskülopatidir. [1,2] Primer SS'de; Tip A (Ro) ve Tip B (La) plazmada yükselen otoantikorlardır. Erken dönemde %20-30, geç dönemde %62-80 oranında pozitif bulunurlar.…”