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Clinical Reviews in Allergy & Immunology

, Volume 53, Issue 3, pp 357–370 | Cite as

Cutaneous and Mucosal Manifestations of Sjögren’s Syndrome

  • Elena Generali
  • Antonio Costanzo
  • Carlo Mainetti
  • Carlo SelmiEmail author
Article

Abstract

Sjögren’s syndrome is currently considered an “autoimmune epithelitis,” as exocrine glands, especially salivary and lacrimal, are progressively destructed by an immune-mediated process associated with specific serum autoantibodies and local lymphocyte infiltrate. Xerostomia remains a key complain in patients with Sjögren’s syndrome but should be evaluated also for other causes such as xerogenic medications, followed by radiation and chemotherapy for head and neck cancers, hormone disorders, infections, or other connective tissue diseases. Further, xerophtalmia (also known as dry eye) frequently associated with keratoconjunctivitis sicca cumulatively affects approximately 10–30% of the general population with increasing incidence with age and is more frequently secondary to non-autoimmune diseases. On the other hand, numerous patients with Sjögren’s syndrome manifest signs of systemic dryness involving the nose, the trachea, the vagina, and the skin, suggesting that other glands are also affected beyond the exocrine epithelia. Skin involvement in Sjögren’s syndrome is relatively common, and various manifestations may be present, in particular xeroderma, eyelid dermatitis, annular erythema, and cutaneous vasculitis. Additional skin non-vasculitic manifestations include livedo reticularis which may occur in the absence of vasculitis, and localized nodular cutaneous amyloidosis possibly representing lymphoproliferative diseases related to Sjögren’s syndrome. The treatment of skin and mucosal manifestations in Sjögren’s syndrome is similar regardless of the cause, starting from patient education to avoid alcohol and tobacco smoking and to pursue dental hygiene. In conclusion, a strict collaboration between the dermatologist and the rheumatologist is essential in the adequate management of Sjögren’s syndrome skin and mucosal manifestations.

Keywords

Autoimmunity Connective tissue disease Sicca syndrome Vasculitis Antinuclear antibody B cell 

Abbreviations

SjS

Sjögren’s syndrome

SLE

Systemic lupus erythematosous

RA

Rheumatoid arthritis

SSc

Systemic sclerosis

PBC

Primary biliary cholangitis

HLA

Human leukocyte antigen

IRF5

Interferon regulatory factor 5

STAT4

Signal transducer and activator of transcription 4

IL

Interleukin

IFN

Interferon

TNF

Tumor necrosis factor

Th

T helper

SGEC

Salivary gland epithelial cells

BEC

Biliary epithelial cells

APC

Antigen-presenting cells

TGFbeta

Transforming growth factor beta

NK

Natural killer

IgG

Immunoglobulin G

IgM

Immunoglobulin M

BAFF

B cell-activating factor

ANA

Antinuclear antibodies

PAMPs

Pathogen-associated molecular patterns

PRRs

Pattern recognition receptors

TLR

Toll-like receptors

NOD

Nucleotide-binding oligomerization domain

RF

Rheumatoid factor

ACA

Anti-centromere antibodies

CENP

Centromeric protein

AMA

Anti-mitocondrial antibodies

anti-CCP

Anti-cyclic citrullinated peptide

Fc

Fragment crystallizable region

TBUT

Tear breakup time

scLE

Subacute cutaneous lupus erythematosus

ESSDAI

European League Against Rheumatism Sjögren’s syndrome disease activity index

AD

Atopic dermatitis

AE

Annular erythema

DM

Dermatomyositis

EULAR

European League Against Rheumatism

ANCA

Anti-neutrophil cytoplasmic antibody

HCQ

Hydroxychloroquine

DMARDs

Disease-modifying antirheumatic drugs

BLys

B lymphocyte stimulator

IVIg

Intravenous immunoglobulins

Notes

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Funding

None.

Ethical Approval and Informed Consent

No ethics issues are raised relevant to this review article.

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Copyright information

© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  1. 1.Division of Rheumatology and Clinical ImmunologyHumanitas Research HospitalMilanItaly
  2. 2.Division of DermatologyHumanitas Research HospitalMilanItaly
  3. 3.Humanitas UniversityMilanItaly
  4. 4.Dermatologia Ente Ospedaliero Cantonale, Ospedale Regionale BellinzonaBellinzonaSwitzerland
  5. 5.BIOMETRA DepartmentUniversity of MilanMilanItaly

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