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Sjögren Syndrome

BASICS

  • Definition:
  • Chronic autoimmune disorder, lymphocytic infiltration of exocrine glands (especially lacrimal/salivary), causing decreased gland function.
  • Presents with sicca symptoms: dry eyes (xerophthalmia), dry mouth (xerostomia), parotid enlargement
  • Extraglandular manifestations: arthralgia, myalgia, Raynaud, pulmonary/GI disease, cytopenias, lymphadenopathy, vasculitis, renal tubular acidosis, lymphoma, CNS & PNS involvement
  • Variants:
  • Primary: Not associated with other disease (HLA-DRB10301, 1501)
  • Secondary: With another autoimmune disorder (RA most common, also SLE, scleroderma)

EPIDEMIOLOGY

  • Annual incidence: ~4/100,000
  • Female:Male = 9:1
  • Onset: 4th–5th decade
  • US prevalence: 1–4 million

ETIOLOGY & PATHOPHYSIOLOGY

  • Unknown etiology; viral trigger (EBV, HCV, HTLV-1) suspected in genetically predisposed
  • Pathology: CD4+ T-cell infiltration → cytokine production → B-cell dysregulation → autoantibody production, chronic inflammation, increased B-cell malignancy
  • Genetics: Familial tendency, HLA-DR associations

RISK FACTORS

  • No known modifiable risk factors

PREVENTION

  • No prevention; early diagnosis/treatment prevents complications
  • Oral health providers are key in early detection/management

COMMONLY ASSOCIATED CONDITIONS

  • Secondary SS: RA, SLE, scleroderma, MCTD, PBC, autoimmune thyroiditis, polymyositis, hepatitis C, HIV, vasculitis, cryoglobulinemia, chronic active hepatitis

DIAGNOSIS

2016 ACR/EULAR Criteria (≥4 points from below + symptoms):

  • +Serum anti-SSA/Ro Ab: 3 points
  • Labial salivary gland biopsy with focus score ≥1 foci/4 mm²: 3 points
  • Abnormal ocular staining score ≥5 or van Bijsterveld score ≥4: 1 point
  • Schirmer test ≤5 mm/5 min: 1 point
  • Unstimulated salivary flow rate ≤0.1 mL/min: 1 point

Exclusion: Prior head/neck radiation, active hepatitis C (PCR+), AIDS, sarcoidosis, amyloidosis, GVHD

HISTORY

  • Ocular: dry, burning, scratchy eyes, sandy/gritty sensation, frequent use of artificial tears
  • Oral: dry mouth, difficulty swallowing, dental caries, tongue sticking to depressor, parotid swelling
  • Systemic: arthralgias, myalgias, fatigue, Raynaud, vasculitis, pulmonary/GI/renal/CNS involvement
  • Gynecologic: vaginal dryness, dyspareunia

PHYSICAL EXAM

  • Eyes: decreased tear pool, keratoconjunctivitis sicca, filamentary keratosis
  • Mouth: dry mucosa, reduced saliva, caries (incisor/cervical), dark red tongue
  • Glands: parotid/submandibular enlargement
  • Skin: vasculitic purpura

DIFFERENTIAL DIAGNOSIS

  • Dry eyes: hypovitaminosis A, age, blepharitis, Parkinson, Bell’s palsy, gland infiltration (lymphoma, sarcoid), low estrogen
  • Dry mouth: meds (anticholinergics, antihistamines, antidepressants), dehydration, anxiety, sialadenitis, hepatitis C/HIV, head/neck radiation
  • Salivary swelling: obstruction, infection, neoplasm, IgG4 disease, alcoholism, cirrhosis, DM, anorexia, viral infection

DIAGNOSTIC TESTS

  • Schirmer test (<5 mm/5 min = abnormal)
  • Rose Bengal test: ocular staining/TBUT
  • Minor salivary gland biopsy: gold standard
  • Autoantibodies: ANA+ (95%), RF+ (75%), anti-SSA (56%), anti-SSB (30%)
  • Imaging: salivary scintigraphy, parotid sialography (not in acute), MRI, salivary US (nonhomogeneity = most useful)
  • Labs: CBC, ESR, CRP, urinalysis, ANA, RF, anti-Ro/SSA, anti-La/SSB

TREATMENT

Goals

  • Symptom relief, improved QOL, manage systemic disease

General

  • Avoid meds worsening dryness (anticholinergics, antidepressants)
  • Oral hygiene, dental care, fatigue management

Dry Mouth (Xerostomia)

  • Nonpharmacologic: gustatory/mechanical stimulants (sugar-free, xylitol gum/lozenges), fluoride mouthwashes, artificial saliva
  • Pharmacologic: cevimeline (Evoxac), pilocarpine (Salagen)
  • Not recommended: routine immunosuppressives, HCQ, oral glucocorticoids, rituximab

Dry Eyes (Keratoconjunctivitis Sicca)

  • Nonpharmacologic: preservative-free artificial tears, gels/ointments (night), humidifiers
  • If severe/unresponsive: topical NSAIDs, corticosteroids, cyclosporine (ophthalmology guidance)

MSK Pain

  • Inflammatory: HCQ 1st line
  • Acute pain: short course acetaminophen/NSAID (<7–10 days)
  • If refractory: MTX, short-term corticosteroids, cyclosporine (if severe)

Other

  • Gynecologic dryness: lubricants, moisturizers; vaginal estrogen if refractory
  • Fatigue: exercise; DHEA not effective above placebo
  • Severe extraglandular: cyclophosphamide, mycophenolate, azathioprine

Procedures

  • Punctal plugs: if refractory dry eyes
  • Surgery: rare, for extraglandular severe complications

REFERRALS

  • Rheumatology: systemic/complex disease
  • Ophthalmology: severe ocular symptoms
  • Dental: annual exam + 2x cleanings/year

COMPLEMENTARY & ALTERNATIVE

  • Acupuncture may help xerostomia

ONGOING CARE

  • Monitor for systemic complications, lymphoma, parotid tumor
  • Adjust frequency based on severity

DIET

  • Reduce sugar, avoid non-water drinks between meals, nothing but water 1 hour before bed

PATIENT EDUCATION

  • Most symptoms managed with nonpharmacologic measures (water intake, humidifier, gum, artificial tears, dental care)
  • Smoking cessation

PROGNOSIS

  • Extraglandular involvement = ↓QOL, ↑mortality
  • Primary SS: ↑ risk lymphoma, thyroid cancer

COMPLICATIONS

  • Dental caries, gum disease, dysphagia, sialolithiasis, keratitis, conjunctivitis, ocular surface scarring

ICD-10

  • M35.01: Sjögren syndrome w/ keratoconjunctivitis
  • M35.03: Sjögren syndrome w/ myopathy
  • M35.00: Sjögren syndrome, unspecified

CLINICAL PEARLS

  • Many symptoms can be treated with artificial tears, sugar-free lozenges, lifestyle mods.
  • Coordinate care: rheumatology, ophthalmology, dentistry.
  • Suspect in unexplained lung disease + ANA+.
  • ↑ risk of lymphoma, celiac disease.