From the Guidelines
Treatment for Sjögren's Syndrome and LIP (Lymphocytic Interstitial Pneumonia) should be based on the severity of the disease, with symptomatic patients receiving moderate dose oral corticosteroids, and those with progressive disease being considered for additional therapies such as rituximab, cyclophosphamide, or antifibrotic drugs like nintedanib.
Treatment Options
- First-line treatment: Moderate dose oral corticosteroids, with a dose not to exceed 60 mg daily of prednisone, and a slow taper over weeks-months 1.
- Steroid-sparing agents: Mycophenolate mofetil (MMF) or azathioprine (AZA) can be used as first-line steroid-sparing or adjunct agents, especially in patients who are not able to successfully taper off corticosteroids or experience unfavorable adverse effects 1.
- Second-line therapies: Rituximab, cyclophosphamide, or antifibrotic drugs like nintedanib can be considered in patients with progressive disease or those who do not respond to first-line treatment 1.
- Additional options: Calcineurin inhibitors, such as cyclosporine or tacrolimus, can be considered in patients who are intolerant to initial maintenance therapy, although there is no evidence to support their superiority in patients who fail first-line therapy 1.
Disease Severity Assessment
- Moderate disease: Shortness of breath on exercise (NYHA II) or pulmonary function tests (PFTs) restricted to forced vital capacity (FVC) between 60% and 80% predicted or diffusing capacity of the lungs for carbon monoxide (DLCO) between 40% and 70% predicted 1.
- Severe/high disease: Shortness of breath at rest (NYHA III, IV) or PFTs with FVC < 60% predicted or DLCO < 40% predicted 1.
Monitoring and Follow-up
- Close follow-up: Required for all pharmacological treatments, with regular assessment of disease progression and adjustment of treatment as needed 1.
- Pulmonary function tests: Should be performed at the time of initial evaluation and repeated every 3 to 6 months, especially in the first 1 to 2 years, to monitor disease progression 1.
From the Research
Treatment for Sjögren's Syndrome and LIP
The treatment for patients with Sjögren's syndrome and Lymphocytic Interstitial Pneumonia (LIP) typically involves:
- Corticosteroid therapy as the mainstay of treatment for Interstitial Lung Disease (ILD) in Sjögren's syndrome 2
- Immunosuppressive drugs, which may be used in addition to corticosteroids, although their use needs to be determined 2
- Symptomatic and supportive measures for airway disease, which may include treatment for dryness and/or inflammation of the airways 2
- Rituximab (anti-CD20) as a promising treatment for LIP, although data from controlled trials are still lacking 3, 4
Treatment Outcomes
Studies have shown that:
- Most patients with LIP and Sjögren's syndrome experience improvement or stabilization of their pulmonary condition after therapy 5
- Treatment with corticosteroids and immunosuppressants can lead to satisfactory results in some cases 4
- Asymptomatic patients with abnormal imaging or patients with mild severity may require careful consideration of treatment decisions, weighing the risk of immunosuppression against the risk of active disease 6
Diagnostic Considerations
Diagnosis of LIP in patients with Sjögren's syndrome may involve: