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Background
- Sjögren syndrome is the second commonest autoimmune rheumatic dysfunction characterised by exocrine gland dysfunction and dryness of mucosal surfaces (sicca signs)
- Estimated population-based prevalence of 0.1%-4.8% worldwide
- The reason for Sjögren syndrome is unknown, however probably attributable to mixture of genetic, environmental (viruses akin to cytomegalovirus and Epstein-Barr virus), and hormonal components
- Often impacts eyes and mouth, however might have an effect on different mucosal surfaces together with the nostril, pharynx, larynx, and vagina
- Illness manifestations past the salivary and lacrimal glands (extraglandular manifestations) are frequent; might have an effect on the musculoskeletal, gastrointestinal, pulmonary, neurologic, renal, and cardiovascular methods
- Could happen as a major dysfunction (major Sjögren syndrome) however about one-third of sufferers have one other underlying autoimmune situation (secondary Sjögren syndrome) akin to rheumatoid arthritis, systemic lupus erythematosus, scleroderma, or hypothyroidism
Pathogenesis
- Autoimmune response triggered by environmental stimulus (akin to viral an infection) in genetically vulnerable affected person might result in lymphocytic infiltration of exocrine glands with degeneration of acinar gland, necrosis, atrophy, and reducing lacrimosalivary perform
- Systemic illness thought of expression of autoimmune epithelitis
- Vasculitis and/or immune advanced deposition and complement activation might play an element in pathogenesis
Proceed Studying
Historical past
- Current sickness
- Could have variable illness course with broad spectrum of scientific manifestations
- Most sufferers current with insidious onset of ≥1 of the next:
- Glandular sicca signs, normally xerophthalmia (dry eyes), xerostomia (dry mouth), and dry pores and skin
- Nonspecific signs, akin to fatigue (in about 70%-80%), sleep disturbances, nervousness, and/or melancholy
- Organ-specific signs attributable to systemic manifestation, akin to musculoskeletal, pulmonary, and/or gastrointestinal signs
- Extraglandular manifestations are frequent, and will embrace:
- Musculoskeletal (myalgia, stiffness, weak point) reported in as much as 90%
- Constitutional (fever, evening sweats, and/or involuntary weight reduction are reported in 9%)
- Gastrointestinal (swallowing problems in over 60%)
- Pulmonary manifestations akin to airway illness, interstitial lung illness (reported in 9%-22%)
- Neurologic manifestations (peripheral neuropathies reported in 5%-21%)
- Hematologic manifestations akin to anemia and hypogammaglobulinemia (reported to happen in 20%)
- Cutaneous manifestations akin to vasculitis or purpura (reported to happen in 10%)
- Renal manifestations (reported to happen in as much as 5%)
- Cardiovascular manifestations (unusual)
- Lymphoma (reported to happen in 5%-7%)
- Medical historical past
- Ask about private or household historical past of autoimmune ailments
Bodily
- Carry out thorough bodily examination to guage for attainable extraglandular manifestations
- Bodily examination findings related to glandular sicca signs embrace:
- HEENT (Head, Eyes, Ears, Nostril, Throat)
- Eye examination
- Bacterial conjunctivitis or bacterial interstitial keratitis
- Corneal melting/ulcers/perforation
- Oral examination
- Lobulated or depapillated crimson tongue
- A number of dental caries/periodontal illness
- Tenderness and/or swelling in salivary glands
- Eye examination
- HEENT (Head, Eyes, Ears, Nostril, Throat)
Prognosis
- There aren’t any universally agreed on diagnostic standards for Sjögren syndrome, however analysis must be suspected in sufferers with sicca signs and ≥1 of the next:
- Constructive blood check for anti-Ro (anti–Sjögren’s-syndrome-related antigen [anti-SS-A]) and anti-La (anti-SS-B) antibodiesPositive salivary gland biopsy with proof of power inflammatory infiltrate in exocrine glands
- Nonspecific and/or organ-specific signs indicative of systemic manifestations
- Differential analysis
- In older sufferers, sicca signs could also be attributable to age-related atrophy of secreting tissue
- Different causes of dry eye:
- Allergic conjunctivitis
- Aqueous tear-deficient dry eye attributable to impaired/dysfunctional lacrimal gland
- Blepharitis
- Hyperevaporative dry eyes
- Rosacea
- Different causes of dry mouth embrace:
- Continual viral infections
- Decreased salivary move attributable to sarcoidosis
- Dehydration
- Diabetes
- Mouth respiration attributable to obstructed nasal passages
- Salivary gland irradiation
- Medication which will trigger sicca signs embrace:
- Alpha-1 and alpha-2 antagonists
- Anticholinergic medicine
- Antihistamines
- Benzodiazepines
- Beta blockers
- Diuretics
- isotretinoin
- Nicotine
- Opioids
- Phenothiazines
- Selective serotonin reuptake inhibitors
- Sympathomimetic medicine
- Tricyclic antidepressants
- Different causes of swollen parotid glands embrace:
- Acute suppurative sialadenitis
- Lymphoma
- Mumps
- Sarcoidosis
- Tuberculosis
Testing
- Preliminary testing might embrace:
- Schirmer check (assesses tear perform)
- Place sterile paper strip beneath decrease eyelid for five minutes
- Constructive check outlined as moistened space ≤5 mm
- Corneal staining with colorants (rose bengal, fluorescein) to evaluate harm to tear meniscus from desiccation to conjunctival epithelium
- Unstimulated entire salivary move assortment to evaluate oral dryness
- Schirmer check (assesses tear perform)
- Blood and serum checks
- No single check can present definitive analysis however might assist decide underlying trigger
- Most sufferers show elevated erythrocyte sedimentation fee (ESR) and cytopenias
- Generally discovered antibodies in Sjögren syndrome embrace:
- Anti-Ro (anti-SS-A) and anti-La (anti-SS-B) antibodies (constructive in 50%-70%)
- Rheumatoid issue (RF) (constructive in 32%-90%)
- Antinuclear antibodies (ANA) (constructive in 55%-97%)
- Polyclonal hypergammaglobulinemia
- Elevated immunoglobulin g and decreased immunoglobulin A
- Imaging
- Scintigraphy: might present delayed uptake, diminished focus, and/or delayed tracer excretion
- Sialography: might present diffuse sialectasia (punctuate, cavitary, or harmful sample) with out main duct obstruction
- Ultrasound to differentiate major Sjögren syndrome from sicca syndrome
- Biopsy of salivary gland
- Could also be indicated in sufferers with adverse anti-RO/LA antibodies
- Pathologic hallmark is power inflammatory infiltrate in exocrine glands
- Salivary gland epithelial cells might show alterations in cell adhesion/form
Administration
- No recognized remedy for Sjögren syndrome — therapy aim is symptom reduction and prevention of issues
- Therapy for sicca signs
- For ocular dryness (xerophthalmia)
- Use preservative-free synthetic tears or ocular gels/ointments as first line therapy
- Cyclosporine ophthalmic answer or emulsion could also be indicated in sufferers with extreme signs
- Systemic pilocarpine and cevimeline might enhance sicca signs
- Punctual occlusion (plugs, cauterization, or surgical procedure)
- Could also be indicated in sufferers with extreme dry eye signs refractory to medicines
- Punctal plugging consists of non permanent or everlasting occlusion of 1 or each puncta with a view to retain tears on the ocular floor by blocking their drainage
- For ocular dryness (xerophthalmia)
- For oral dryness (xerostomia)
- Mechanical stimulation, gustatory stimulation, and/or saliva substitutes thought of first line of therapy
- Systemic therapy with pilocarpine or cevimeline to enhance salivary move
- Intraoral electrostimulation system
- Frequent dental exams, antimicrobial mouth rinses, and each day fluoride use might assist stop caries
- Administration of extraglandular manifestations
- Glucocorticoids
- Needs to be at minimal dose and time wanted to manage energetic systemic illness
- Could downregulate inflammatory processes in salivary and lacrimal glands
- Lengthy-term use must be prevented attributable to threat of adversarial results
- Glucocorticoids
- Immunosuppressive and immunomodulatory brokers
- Take into account immunosuppressive use as glucocorticoid-sparing brokers
- Take into account rituximab
- For adults with major Sjögren syndrome and suboptimal response or toxicity to straightforward remedy, incapacity to taper corticosteroids, and any of the next:
- Cryoglobulinemia related to vasculitis
- Inflammatory arthritis
- Peripheral neuropathy
- Pulmonary illness
- Extreme parotid swelling
- Vasculitis
- May additionally be thought of (if standard remedy inadequate) for therapy of
- Keratoconjunctivitis sicca
- Xerostomia if some proof of residual salivary manufacturing
- For adults with major Sjögren syndrome and suboptimal response or toxicity to straightforward remedy, incapacity to taper corticosteroids, and any of the next:
Observe-up
- Recommended frequency
- Yearly in sufferers with steady illness/restricted to mucosal surfaces
- Each 6 months in sufferers with systemic, extraglandular manifestations
- Each 3 months in sufferers with end-organ harm
- Medical examination to evaluate:
- Mouth and eyes for proof of issues
- For presence of lymphadenopathy
- Enlargement of parotid and submandibular glands, liver, or spleen
- Annual laboratory checks ought to embrace:
- Full blood depend
- Erythrocyte sedimentation fee
- Renal and liver perform checks
- Immunologic checks not normally indicated in routine follow-up
Problems
- Problems from glandular sicca signs might embrace:
- Lack of protecting and antimicrobial results of saliva might improve threat for
- Problems of xerophthalmia (extreme dry eye) might embrace
- Destruction of conjunctival epithelium
- Extraglandular illness manifestations might lead to a extremely variable vary of issues attributable to quite a few attainable systemic manifestations and serologic abnormalities
Kendra Church MS, PA-C, is a doctor assistant at Dana-Farber Most cancers Institute/Brigham & Ladies’s Hospital, and can also be a senior scientific editor for DynaMed, an evidence-based, point-of-care database.
References
- Tincani A, Andreoli L, Cavazzana I, et al. Novel points of Sjögren’s syndrome in 2012. BMC Med. 2013;11:93. doi: 10.1186/1741-7015-11-93
- Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, Bosch X. Main Sjögren syndrome. BMJ. 2012;344:e3821. doi:10.1136/bmj.e3821
- Vivino FB. Sjögren syndrome: scientific points. Clin Immunol. 2017;182:48-54. doi:10.1016/j.clim.2017.04.005
- Carsons SE, Vivino FB, Parke A, et al. Therapy pointers for rheumatologic manifestations of Sjögren syndrome: use of biologic brokers, administration of fatigue, and inflammatory musculoskeletal ache. Arthritis Care Res (Hoboken). 2017;69(4):517-527. doi:10.1002/acr.22968
- Leone MC, Alunno A, Cafaro G, et al. The scientific spectrum of major Sjögren syndrome: past exocrine glands. Reumatismo. 2017 Sep 21;69(3):93-100. doi:10.4081/reumatismo.2017.1032
- Vivino FB. Sjögren syndrome: scientific points. Clin Immunol. 2017;182:48-54. doi:10.1016/j.clim.2017.04.005
- Alunno A, Carubbi F, Bartoloni E, Cipriani P, Giacomelli R, Gerli R. The kaleidoscope of neurological manifestations in major Sjögren syndrome. Clin Exp Rheumatol. 2019;37 Suppl 118(3):192-198.
- Mariette X, Criswell LA. Main Sjögren syndrome. N Engl J Med. 2018;378(10):931-939. doi:10.1056/NEJMcp1702514
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