Elsevier

Clinics in Dermatology

Volume 18, Issue 5, September–October 2000, Pages 589-600
Clinics in Dermatology

Sjogren’s syndrome:

https://doi.org/10.1016/S0738-081X(00)00135-8Get rights and content

Section snippets

Diagnostic issues

In 1932, Henrik Sjögren reported the triad of keratoconjunctivitis sicca (KCS), xerostomia, and rheumatoid arthritis. The clinical spectrum of SS as a systemic autoimmune disease was further defined by Bloch et al.1 in 1956. In patients with extreme manifestations there is good agreement on diagnosis among clinicians, but the diagnosis in patients with milder sicca symptoms has remained controversial owing to the absence of good noninvasive methods for documenting xerostomia. The demonstration

Pathogenesis of Sjogren’s syndrome

The epithelial cells lie upon a specific extracellular matrix including collagens, laminins, vitronectin, and nonmatrix proteins. Cell-matrix interactions are important for cellular functions of the epithelial cell, including response to growth factor signals and ability for cellular regeneration.42 The epithelial cells express a family of specific receptors including integrins to bind these matrix proteins, as well as receptors for cyclic AMP (cAMP), growth factors, estrogens, and cytokines.43

Therapy of Sjogren’s syndrome

The characteristic symptoms of SS patients involve dry, painful eyes and mouth. For dry eyes, the initial therapy is artificial tears on a regular basis. It is a common problem to wait for symptoms and then play “catch up.” The patient needs to evaluate the needs in the day ahead (ie. dry winds, low humidity such as an airplane) and adjust the tear use in anticipation. Different brands of artificial tears have distinct characteristics in terms of their viscosity (ie. the more viscous tears last

First page preview

First page preview
Click to open first page preview

References (151)

  • H. Saari et al.

    Salivary mucous glycoprotein MG1 in Sjogren’s syndrome

    Clin Chim Acta

    (1997)
  • B.L. Slomiany et al.

    Salivary mucins in oral mucosal defense

    Gen Pharmacol

    (1996)
  • C. Main et al.

    Human recombinant interleukin-1-beta suppresses acetylcholine release from rat myenteric plexus

    Gastroenterology

    (1993)
  • G. Lu et al.

    Tumor necrosis factor-alpha and interleukin-1 induce activation of MAP kinase and SAP kinase in human neuroma fibroblasts

    Neurochem Int

    (1997)
  • I.L. Campbell

    Neuropathogenic actions of cytokines assessed in transgenic mice

    Int J Dev Neurosci

    (1995)
  • J. Tornwall et al.

    Salivary gland acinar epithelial cells are deficient in their protein kinase C expression in Sjogren’s syndrome

    Lancet

    (1997)
  • L. Royce et al.

    Human neoplastic submandibular intercalated duct cells express an acinar phenotype when cultured on basement membrane matrix

    Differentiation

    (1993)
  • A.N. Akbar et al.

    Cellular environments and apoptosisTissue microenvironments control activated T-cell death

    Immunol Today

    (1997)
  • H. Hyde et al.

    Upregulation of intracellular glutathione by fibroblast-derived factor(s)Enhanced survival of activated T cells in the presence of low Bcl-2

    Blood

    (1997)
  • D.M. Janz et al.

    Suppression of apoptosis by gonadotropin, 17beta-estradiol, and epidermal growth factor in rainbow trout preovulatory ovarian follicles

    Gen Comp Endocrinol

    (1997)
  • C.P. Robinson et al.

    Genetically programmed development of salivary gland abnormalities in the NOD (nonobese diabetic)-scid mouse in the absence of detectable lymphocytic infiltrationA potential trigger for sialoadenitis of NOD mice

    Clin Immunol Immunopathol

    (1996)
  • A.N. Theofilopoulos

    The basis of autoimmunityPart I. Mechanisms of aberrant self-recognition

    Immunol Today

    (1995)
  • K.J. Bloch et al.

    Sjögren’s syndromeA clinical, pathological and serological study of 62 cases

    Medicine (Baltimore)

    (1956)
  • T.E. Daniels et al.

    Association of patterns of labial salivary gland inflammation with keratoconjunctivitis sicca. Analysis of 618 patients with suspected Sjogren’s syndrome

    Arthritis Rheum

    (1994)
  • C. Vitali et al.

    The European Community Study Group on diagnostic criteria for Sjogren’s syndrome. Sensitivity and specificity of tests for ocular and oral involvement in Sjogren’s syndrome

    Ann Rheum Dis

    (1994)
  • C. Jorgensen et al.

    Sicca syndrome associated with hepatitis C virus infection

    Arthritis Rheum

    (1996)
  • C. Vitali et al.

    The European classification criteria for Sjogren’s syndrome (SS)Proposal for modification of the rules for classification suggested by the analysis of the receiver operating characteristic (ROCS) curve of the criteria performance

    J Rheum

    (1997)
  • M.E. Stern et al.

    The pathology of dry eyeThe interaction between the ocular surface and lacrimal glands

    Cornea

    (1998)
  • C.S. Cho et al.

    Clinical significances of antibodies to Ro/SS-A autoantigens and its subtypes in primary Sjogren’s syndrome

    Korean J Intern Med

    (1997)
  • F. Gemignani et al.

    Peripheral neuropathy associated with primary Sjogren’s syndrome

    J Neurol Neurosurg Psychiatry

    (1994)
  • M. Hebbar et al.

    Participation of cryoglobulinaemia in the severe peripheral neuropathies of primary Sjogren’s syndrome

    Ann Med Interne (Paris)

    (1995)
  • R. Kyle et al.

    Benign hyperglobuliemic purpura of Waldenstrom

    Medicine (Baltimore)

    (1971)
  • R.I. Fox et al.

    Characterization of a cross-reactive idiotype in Sjögren’s syndrome

    Scand J Rheumatol

    (1986)
  • T.T. Provost et al.

    Anti-Ro(SS-A) antibody positive Sjogren’s/lupus erythematosus overlap syndrome

    Lupus

    (1997)
  • E. Alexander et al.

    Sjögren’s syndrome. Association of cutaneous vasculitis with central nervous system disease

    Arch Dermatol

    (1987)
  • M. Konishi et al.

    Mixed-cryoglobulinemia associated with cutaneous vasculitis and pulmonary symptoms

    J Intern Med

    (1997)
  • C. Ferri et al.

    Mixed cryoglobulinaemiaA cross-road between autoimmune and lymphoproliferative disorders

    Lupus

    (1998)
  • P.A. Merkel et al.

    Prevalence of antineutrophil cytoplasmic antibodies in a large inception cohort of patients with connective tissue disease

    Ann Intern Med

    (1997)
  • M. Navarro et al.

    Anti-endothelial cell antibodies in systemic autoimmune diseasesPrevalence and clinical significance

    Lupus

    (1997)
  • R.A. Asherson et al.

    Antiphospholipid antibodies and HLA associations in primary Sjögren’s syndrome

    Ann Rheum Dis

    (1992)
  • I.A. Grant et al.

    Peripheral neuropathy associated with sicca complex

    Neurology

    (1997)
  • E.L. Alexander et al.

    Magnetic resonance imaging of cerebral lesions in patients with the Sjögren’s syndrome

    Ann Intern Med

    (1988)
  • R.I. Fox

    Sjogren’s syndrome

    Curr Opin Rheumatol

    (1995)
  • R.L. Ochs et al.

    cDNA cloning and characterization of a novel nucleolar protein

    Mol Biol Cell

    (1996)
  • D.R. Singer et al.

    CryoglobulinaemiaClinical features and response to treatment [published erratum appears in Ann Med Interne (Paris) 1986;137(5):436]

    Ann Med Intern

    (1986)
  • G.M. Mody et al.

    Rheumatologic manifestations of gastrointestinal disorders

    Curr Opin Rheumatol

    (1998)
  • Fox RI, Wilson C. Sjögren’s syndrome: The association of interstitial nephritis and keratoconjunctivitis. Diseases...
  • J.L. Pablos et al.

    Subcutaneous nodular amyloidosis in Sjögren’s syndrome

    Scand J Rheumatol

    (1993)
  • R.M. Ricci et al.

    Primary anetoderma

    Cutis

    (1998)
  • C. Jubert et al.

    Sjogren’s syndrome and cutaneous B cell lymphoma revealed by anetoderma

    Arthritis Rheum

    (1993)
  • Cited by (0)

    The research of R.I.F. was supported by NIH grant M01-RR000831 and the Department of Academic Affairs of Scripps Institute for Medicine. Additional support for postdoctoral fellows (J.H. and C.C.) was provided by grants from the Hennings Memorial Trust, and the Florence, Scripps-Stedham, Kovler and Ramsdell Foundations.

    View full text