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Sjögren's disease

Definition
SD is an autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, leading to glandular dysfunction, particularly of the salivary and lacrimal glands.
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Pathophysiology
SD is hypothesized to be triggered by environmental factors, such as viral infections (EBV, human herpes virus 6, human T cell lymphotropic virus 1, HCV) and smoking in genetically predisposed individuals (HLA class I, class II and class III genes).
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Epidemiology
The estimated incidence of SD is 3.9 per 100,000 person-years in the US.
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Disease course
The activation of mucosal epithelial cells secondary to lymphocytic infiltration leads to the activation of innate and acquired immune systems by secreting autoantibodies. These autoantibodies form immune complexes that maintain and amplify the production of interferon alpha, resulting in a cycle of immune system activation that ultimately causes tissue damage. The spectrum of associated end-organ disease includes exocrine glandular dysfunction, ILD, lymphoma, interstitial nephritis, cryoglobulinemia, arthralgias, cutaneous lupus, peripheral neuropathies, and cerebral vasculitis.
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Prognosis and risk of recurrence
Lymphoma, when it occurs in patients with SD, has a high mortality rate, with a disease-specific standardized mortality ratio of 7.89.
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Key sources
The following summarized guidelines for the evaluation and management of Sjögren’s disease are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023; 2021), the Brazilian Society of Rheumatology (BSR 2022), the European League Against Rheumatism (EULAR 2022; 2020), the European Respiratory Society (ERS 2022), the Royal College of Ophthalmologists (RCOphth 2020), and the Sjögren's Syndrome Foundation (SSF 2017).
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Guidelines

1.Screening and diagnosis

Screening in patients with lung disease: obtain clinical and serologic evaluation for SD in patients with uncharacterized ILD, diffuse cystic lung disease, or pulmonary lymphoma.
A
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2.Classification and risk stratification

Severity assessment: as per BSR 2022 guidelines, use the ESSDAI as a measurement tool for diagnosing and evaluating the activity of systemic manifestations of SD.
B

3.Diagnostic investigations

History and physical examination
As per BSR 2022 guidelines:
Assess the inflammatory arthralgia and/or non-erosive arthritis in patients with primary Sjögren's syndrome to classify according to the number and location of joints involved. Confirm suspected arthritis by physical examinations and, if necessary, imaging tests.
A
Recognize that respiratory impairment in primary Sjögren's syndrome is frequent, polymorphic, and may be associated with decreased QoL, development of lymphoma, and worse prognosis. Assess all patients for the presence of respiratory signs and symptoms at diagnosis and follow-up visits.
A

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  • Serologic biomarkers

  • Chest radiography

  • Echocardiography

  • CT

  • CT pulmonary angiogram

  • Ventilation-perfusion scan

  • Pulmonary function testing

  • PET

  • Screening for renal involvement

  • Screening for chronic infections

4.Diagnostic procedures

Bronchoalveolar lavage
Do not perform routine bronchoscopy with BAL in patients with SD having respiratory symptoms.
D
Consider performing bronchoscopy with BAL in patients with SD having respiratory symptoms determined on a case-by-case basis and limited to special circumstances, such as the need to:
rule out infectious etiologies, especially in patients on immune suppression
rule out endobronchial abnormalities such as amyloidosis in patients with chronic cough not otherwise responsive to treatment
distinguish between other etiologies of sicca symptoms such as sarcoidosis
B

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  • Lung biopsy

5.Respiratory support

Oxygen therapy
Initiate long-term oxygen therapy in patients with SD and suspected ILD and clinically significant resting hypoxemia (defined by resting oxygen saturation < 88%, PaO2 < 55 mmHg or < 60 mmHg with complication of chronic hypoxemia such as cor pulmonale).
B
Evaluate the need for supplemental oxygen in patients with SD-associated ILD considering air travel.
B

6.Medical management

Management of xerostomia
As per EULAR 2020 guidelines:
Obtain baseline evaluation of salivary gland function before initiating treatment for oral dryness.
B
Consider offering the following options, according to salivary gland dysfunction, as first-line treatment for oral dryness:
Situation
Guidance
Mild
Nonpharmacological stimulation
B
Moderate
Pharmacological stimulation
C
Severe
Saliva substitution
B

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  • Management of keratoconjunctivitis sicca

  • Management of fatigue

  • Management of musculoskeletal pain (first-line)

  • Management of musculoskeletal pain (second-line)

  • Management of musculoskeletal pain (third-line)

  • Management of systemic manifestations

  • Management of dry cough

  • Management of vocal cord cystic lesions

  • Management of small airway disease

  • Management of bronchiectasis

  • Management of ILD (systemic corticosteroids)

  • Management of ILD (mycophenolate mofetil and azathioprine)

  • Management of ILD (rituximab, calcineurin inhibitors, cyclosporine, tacrolimus)

  • Management of ILD (antifibrotic agents)

  • Management of ILD (cyclophosphamide)

7.Nonpharmacologic interventions

Exercise and self-care: provide education regarding self-care measures, including advice about exercise to reduce fatigue in patients with SD.
A

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  • Smoking cessation

  • Air humidification

  • Pulmonary rehabilitation

  • Voice therapy

8.Surgical interventions

Vocal cord surgery: consider performing surgical resection in patients with SD and symptomatic vocal cord cystic lesions ("bamboo nodules") if initial less aggressive measures fail, with consultation by a laryngologist with experience in SD.
C

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  • Lung transplantation

9.Patient education

Patient education: educate patients and caregivers/family about signs and symptoms of pneumothorax and instruct to seek immediate medical attention if they experience signs or symptoms because patients with SD and cystic lung disease might have an increased risk of pneumothorax.
B

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  • Air travel

  • Medication-related adverse effects

10.Preventative measures

Routine immunizations: as per ACR 2023 guidelines, consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in ≥ 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications.
C
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  • Prophylaxis for P. jirovecii pneumonia

11.Follow-up and surveillance

Monitoring of patients on rituximab: monitor for the following adverse effects in patients receiving rituximab:
infusion reactions
tumor lysis syndrome in patients with non-Hodgkin's lymphoma
progressive multifocal leukoencephalopathy
hepatitis B reactivation with possible fulminant hepatitis
severe mucocutaneous reactions
infections
bowel obstruction and perforation
cardiac arrhythmias and angina
cytopenias
serious bacterial, viral, or fungal infections
B

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  • Monitoring of patients on TNFi

  • Monitoring of patients on hydroxychloroquine/chloroquine

  • Monitoring for pulmonary function

  • Monitoring for lymphoproliferative disorders