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Sjögren's disease

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Updated 2024 BSR guidelines for the diagnosis and management of Sjögren's disease.

Background

Overview

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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Guidelines

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 British Society for Rheumatology (BSR 2024), the American College of Rheumatology (ACR 2023), the European League Against Rheumatism (EULAR 2023,2020), the Brazilian Society of Rheumatology (BSR 2022), the European Respiratory Society (ERS 2022), the Consensus Expert Panel ...
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Screening and diagnosis

Indications for screening: as per PDSS-CEP 2021 guidelines, obtain clinical and serologic evaluation for SD in patients with uncharacterized ILD, diffuse cystic lung disease, or pulmonary lymphoma.
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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.
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Diagnostic investigations

History and physical examination
As per BSR 2024 guidelines:
Obtain baseline assessment with a thorough clinical and serological evaluation in patients with SD to inform the risk of development of extraglandular features and disease progression.
B
Obtain early further evaluation in patients with Sjögren's syndrome presenting with new salivary gland swelling or other symptoms suggestive of lymphoma.
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  • Serologic biomarkers

  • Salivary gland ultrasound

  • CXR

  • Echocardiography

  • Chest CT

  • CTPA

  • V/Q scan

  • Pulmonary function testing

  • PET

  • Screening for renal involvement

  • Screening for chronic infections

  • Screening for comorbidities

Diagnostic procedures

Bronchoalveolar lavage
As per PDSS-CEP 2021 guidelines:
Do not perform routine bronchoscopy with bronchoalveolar lavage in patients with SD having respiratory symptoms.
D
Consider performing bronchoscopy with bronchoalveolar lavage 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
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  • Lung biopsy

  • Salivary gland biopsy

  • Lacrimal gland biopsy

Respiratory support

Oxygen therapy
As per PDSS-CEP 2021 guidelines:
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.
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Medical management

General principles: as per BSR 2024 guidelines, consider administering short-term systemic corticosteroids for specific indications, but not routinely in the management of SD.
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  • Management of xerostomia

  • Management of parotitis

  • Management of keratoconjunctivitis sicca

  • Management of vaginal dryness

  • Management of fatigue (nonpharmacological interventions)

  • Management of fatigue (hydroxychloroquine)

  • Management of fatigue (other agents)

  • 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)

Nonpharmacologic interventions

Exercise and self-care: as per SSF 2017 guidelines, provide education regarding self-care measures, including advice about exercise, to reduce fatigue in patients with SD.
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  • Smoking cessation

  • Air humidification

  • Pulmonary rehabilitation

  • Voice therapy

  • Dietary supplements

Surgical interventions

Vocal cord surgery: as per PDSS-CEP 2021 guidelines, 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.
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  • Lung transplantation

Specific circumstances

Pregnant patients: as per BSR 2024 guidelines, initiate low-dose aspirin in patients at high risk of preeclampsia or with a high-risk pregnancy in general.
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Patient education

General counseling: as per BSR 2024 guidelines, provide written information on the manifestations of SD and their management, direct patients with SD to appropriate online resources, and advise accessing local and national support groups.
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  • Air travel

  • Medication-related adverse effects

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.
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  • Prophylaxis for P. jirovecii pneumonia

Follow-up and surveillance

Monitoring of patients on rituximab: as per SSF 2017 guidelines, 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.
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  • Monitoring of patients on TNFi

  • Monitoring of patients on hydroxychloroquine/chloroquine

  • Monitoring for pulmonary function

  • Monitoring for lymphoproliferative disorders