Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations

Abstract

Primary Sjögren's syndrome (1 • SS) is an autoimmune disorder characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth due to lymphocytic infiltrates of lacrimal and salivary glands. However, SS also affects many extraglandular systems. In SS patients, the pattern of extraglandular manifestations may have a close similarity with the vasculitic features seen in SLE patients that are mediated by immune complexes and complement. However, SS patients also have an increased frequency of lymphocytic infiltration into extraglandular tissues, as might be expected by their increased frequency of lymphoma in comparison to SLE patients. For example, SS patients need to be evaluated for interstitial nephritis (in contrast to the glomerulonephritis of SLE) or interstitial pneumonitis (in comparison to pleurisy of SLE). This chapter will focus on the clinical extraglandular manifestations of primary SS that are not specifically covered in other chapters. These extraglandular manifestations have led to a recently introduced "disease activity" and "organ damage index." The recognition of these extraglandular manifestations is important since they have prognostic and therapeutic implications. The differential diagnosis of these extraglandular manifestations includes overlapping features with other autoimmune diseases (particularly systemic lupus erythematosus (SLE), scleroderma, dermatomyositis, celiac sprue, and small-and medium-sized vessel vasculitis), infectious diseases that mimic autoimmune disease (particularly hepatitis C, HIV, syphilis, tuberculosis), and predisposition to drug toxicities that may involve extraglandular organs (particularly skin rashes, nephritis, pneumonitis, myositis, and hematopoietic abnormalities). R.I. Fox ( )

Pulmonary Manifestations of Sjögren’s Syndrome

“…Interstitial lung disease (ILD) refers to a group of lung diseases affecting the interstitium of the lung: alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues (24). Historically, the predominant form of ILD in primary SS was deemed to be lymphocytic interstitial pneumonitis (LIP).…”

Salivary free light chains and salivary immunoglobulins as potential non‐invasive biomarkers in primary Sjögren's syndrome

“…2,3 It is now increasingly being recognized that rather than merely a disease of dryness, pSS is a systemic disease with extraglandular involvement in close to 50%-60% of patients. 4,5 There is therefore an unmet need for cost-effective, sensitive biomarkers that could aid in diagnosis as well as prognosis. In the absence of a clear-cut constellation of symptoms and diagnostic criteria, most physicians depend on classification criteria for the diagnosis.…”

Association between a History of herpes zoster and the risk of Sjögren’s syndrome: a nationwide, population-based, case–control study

“…Because comorbidities may be associated with both herpes zoster and SS, they are considered as potential confounders for the association between herpes zoster and the risk of SS. We used the Charlson Comorbidity Index (CCI) as the general comorbidity index25 26 after excluding connective tissue disease (CTD) to examine the presence of comorbidities including diabetes with diabetic complications, myocardial infarction, congestive heart failure, peripheral vascular disease,27 28 chronic pulmonary disease,29–31 ulcer disease,32 mild liver disease,33 34 moderate or severe liver disease, hemiplegia, moderate or severe renal disease, tumour35 and solid metastatic tumour. Patients were considered to have any comorbidity when they had at least three outpatient visits or one hospitalisation with the corresponding ICD-9-CM code within 1 year before the index date.…”