• Skip to primary navigation
  • Skip to main content
  • Skip to primary sidebar
  • Skip to footer

Johns Hopkins Sjögren’s Center

Show Search
Hide Search
  • Sjögren’s Syndrome Information
    • Diagnosis of Sjögren’s Syndrome
    • Sjögren’s Syndrome Symptoms
    • Treatment of Sjögren’s Syndrome
    • Sjögren’s Syndrome Disease Education Series
  • About the Center
    • Meet Our Team
    • Schedule An Appointment
    • Physician Referral Information
    • Directions
  • Research
  • Support the Center
  • Contact Us
Home / Sjögren’s Syndrome Information / Treatment of Sjögren’s Syndrome / Management of Systemic Features of Sjögren’s Syndrome

Management of Systemic Features of Sjögren’s Syndrome

The treatment of most systemic (so-call “extra-glandular manifestations”) involves a hierarchy of treatments, starting with those appropriate for milder disease and ending with those for more severe disease.

1. Arthritis is initially treated with hydroxychloroquine with or without non-steroidal anti-inflammatory agents. More severe arthritis may require low-dose corticosteroids, methotrexate, leflunomide, TNF antagonists, or rituximab.

2. Fatigue is generally treated in a multidisciplinary fashion, with attention to concomitant depression, fibromyalgia, and sleep disorders. Hydroxychloroquine is often prescribed, but has not been formally tested for this indication. Most studies have shown reduction in the ESR and immunoglobulin levels with hydroxychloroquine therapy, but the clinical impact of this is uncertain. Rituximab showed some benefit in reducing fatigue in a randomized clinical trial.

3. Renal involvement. Distal renal tubular acidosis is treated with alkali (bicarbonate or citrate salts) to control the acidosis and present the formation of renal stones. In patients with impaired renal function or significant proteinuria, a renal biopsy should be performed. The finding of tubulointerstitial nephritis or glomerulonephritis should prompt treatment with a course of corticosteroids as the first line of therapy. The role of long-term steroid-sparing immunosuppressive agents has not been defined. Cryoglobulinemic glomerulonephritis related to SS has a poor prognosis and requires more intensive therapy, often corticosteroids with cyclophosphamide or rituximab.

4. Interstitial lung disease in SS tends to be mild and does not require treatment if there is no significant impairment in forced vital capacity (i.e. >75%) or diffusion capacity (>65%). Corticosteroids are the first-line treatment and may suffice for lymphoid interstitial pneumonia or organizing pneumonia patterns of involvement. Azathioprine, mycophenolate and cyclophosphamide can be used as steroid-sparing agents, particularly if the lung disease progresses with corticosteroid monotherapy.

5. Cutaneous vasculitis may not require pharmacologic treatment when manifested as a mild intermittent purpura. Support stockings and avoidance of prolonged standing may suffice. More severe flares may require moderate dose corticosteroids, usually in a tapering dose. Hydroxychloroquine, colchicine, dapsone, methotrexate, azathioprine, and mycophenolate mofetil have potential utility to control recurrences and minimize corticosteroid exposure. Rituximab is being used increasingly for control of cryoglobulinemic vasculitis in SS. Cyclophosphamide is reserved for refractory cases or those with more serious manifestations.

Receive the Latest News from Johns Hopkins Rheumatology

Receive the Latest News from Johns Hopkins Rheumatology

Join our mailing list to receive the latest news and updates from Johns Hopkins Rheumatology.

Interested In

You have Successfully Subscribed!

Use of this Site

All information contained within the Johns Hopkins Jerome L. Green Sjogren’s Center website is intended for educational purposes only. Physicians and other health care professionals are encouraged to consult other sources and confirm the information contained within this site. Consumers should never disregard medical advice or delay in seeking it because of something they may have read on this website.

Primary Sidebar

Sjögren’s Syndrome Information

  • Sjögren’s Syndrome Overview
  • Symptoms of Sjögren’s Syndrome
  • Diagnosis of Sjögren’s Syndrome
  • Treatment of Sjögren’s Syndrome

Dr. Alan Baer in Chair in Front of Brick Wall Discussing Sjögren’s Syndrome

In this five-part video series on Sjögren’s syndrome, Dr. Alan Baer validates those who suffer from this disease, and informs those who don’t, about the common misconceptions and challenges that Sjögren’s syndrome patients face every day.

Footer

Johns Hopkins Rheumatology

  • Johns Hopkins Rheumatology
  • Johns Hopkins Arthritis Center
  • Johns Hopkins Lupus Center
  • Johns Hopkins Lyme Disease Research Center
  • Johns Hopkins Myositis Center
  • Johns Hopkins Scleroderma Center
  • Johns Hopkins Vasculitis Center

Connect With Us

  • Facebook
  • Twitter
  • YouTube
U.S. News and World Report Rankings Badge

Johns Hopkins Medicine

© 2023 Johns Hopkins Jerome L. Greene Sjogren's Syndrome Center
Patient Privacy