From the Guidelines
Patients with Sjögren's disease have a significantly increased risk of developing Mucosa-Associated Lymphoid Tissue (MALT) lymphoma, with approximately 5-18% of Sjögren's patients developing this malignancy over their lifetime, as reported in the 2021 consensus guidelines for evaluation and management of pulmonary disease in Sjögren's 1.
Epidemiology of MALT Lymphoma in Sjögren's Disease
The epidemiology of MALT lymphoma in Sjögren's disease is characterized by:
- Increased risk of lymphoma development, ranging from 5% to 18% 1
- MALT lymphoma most commonly affects the salivary glands, particularly the parotid glands, though it can also develop in other extranodal sites including the lungs, stomach, and thyroid
- Lymphoproliferative involvement of the lungs can present as non-resolving consolidations, focal nodules, lymphadenopathy, and cystic lesions accompanied by adjacent nodules and may be asymptomatic 1
Management of MALT Lymphoma in Sjögren's Disease
The management of MALT lymphoma in Sjögren's disease typically begins with:
- Careful staging using CT scans, PET-CT, bone marrow biopsy, and endoscopic evaluation when appropriate
- For localized MALT lymphoma (stage I-II), radiation therapy at doses of 24-30 Gy over 2-3 weeks is often the first-line treatment, with complete response rates exceeding 90% 1
- For disseminated disease, systemic therapy options include rituximab monotherapy (375 mg/m² weekly for 4 weeks), which is particularly effective in CD20-positive cases, or rituximab combined with chemotherapy regimens such as bendamustine or CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) 1
Slide with Notes
Here is a suggested slide with notes:
- Title: MALT Lymphoma in Sjögren's Disease
- Bullet points:
- Increased risk of lymphoma development (5-18%)
- MALT lymphoma most commonly affects salivary glands, lungs, stomach, and thyroid
- Lymphoproliferative involvement of the lungs can present as non-resolving consolidations, focal nodules, lymphadenopathy, and cystic lesions
- Management includes careful staging, radiation therapy, and systemic therapy options such as rituximab and chemotherapy
- Image: a diagram or illustration of the salivary glands and lungs, highlighting the common sites of MALT lymphoma involvement
- References: 1, 1
From the Research
Epidemiology of MALT Lymphoma in Sjögren's Disease
- Sjögren's syndrome patients have a 44-fold excess risk for the development of non-Hodgkin's lymphoma, particularly mucosa-associated lymphoid tissue (MALT) lymphoma 2.
- MALT lymphoma is an indolent disease, and prolonged remission has been obtained with different therapeutic approaches 3.
- The majority of MALT lymphomas in Sjögren's syndrome patients are localized in the major salivary glands, but cases of MALT lymphoma in minor salivary glands have also been reported 2.
Management of MALT Lymphoma in Sjögren's Disease
- Treatment of MALT lymphoma in Sjögren's syndrome patients can include "watchful waiting", surgery, radiotherapy, rituximab, or rituximab combined with chemotherapy 4.
- Rituximab has been shown to be effective in treating MALT lymphoma in Sjögren's syndrome patients, with complete response observed in some cases 5, 4, 6.
- The choice of treatment depends on the stage and severity of the disease, as well as the patient's overall health status.
- Patients with high Sjögren's syndrome disease activity may require treatment for both MALT lymphoma and Sjögren's syndrome 4.
Slide Notes
- Title: MALT Lymphoma in Sjögren's Disease
- Bullet points:
- Definition: MALT lymphoma is a type of non-Hodgkin's lymphoma that arises in the mucosa-associated lymphoid tissue.
- Epidemiology: Sjögren's syndrome patients have a 44-fold excess risk for the development of MALT lymphoma.
- Management: Treatment options include "watchful waiting", surgery, radiotherapy, rituximab, or rituximab combined with chemotherapy.
- Prognosis: Prolonged remission has been obtained with different therapeutic approaches.
- Images: Consider adding images of salivary gland biopsies or radiologic scans to illustrate the disease.