Sjögren’s Syndrome: Causes, symptoms, diagnosis and treatment

Sjögren’s Syndrome is an autoimmune disorder that primarily affects the moisture-producing glands, leading to symptoms of dry eyes and dry mouth. The exact cause is unknown but it is believed to involve a combination of genetic, environmental and hormonal factors triggering the immune system to attack the body’s moisture-producing glands.
In an interview with HT Lifestyle, Dr Shailaja Sabnis, Consultant Rheumatologist at Nanavati Max Super Speciality Hospital in Mumbai shared, “Common symptoms include dry eyes, dry mouth, difficulty swallowing, dental cavities, joint pain, fatigue and in some cases, organ involvement affecting the kidneys, lungs, or nervous system. Diagnosis often involves a comprehensive medical history review, physical examination, and specialized tests. Blood tests to check for specific antibodies like anti-SSA (Ro) and anti-SSB (La) antibodies, along with imaging studies like salivary gland or lip biopsy, can aid in confirming the diagnosis.”
According to her, management of Sjögren’s Syndrome aims at relieving symptoms and preventing complications. She revealed, “For dry eyes, artificial tears, prescription eye drops or punctal plugs may be recommended. Similarly, for dry mouth, saliva substitutes, medications to stimulate saliva production, and good oral hygiene are essential. Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) or other medications may help manage joint pain and inflammation. Additionally, a multidisciplinary approach involving rheumatologists, ophthalmologists, dentists, and other specialists is crucial to address systemic complications. Lifestyle modifications, such as staying hydrated and avoiding dry environments, can also ease symptoms. Regular monitoring and a proactive approach in managing symptoms are key components of effective care for individuals with this condition.”
Bringing his expertise to the same, Dr Raghavendra H, Consultant Rheumatologist at Manipal Hospital in Bangalore’s Hebbal, explained, “Sjögren’s Syndrome is an autoimmune disease primarily affecting women between the ages of 40-60 years. It could be primarily an autoimmune disease or be associated with any other autoimmune disease. As an autoimmune disease, it has varied manifestations. The characteristic presentations include dryness of mouth, eyes, and skin. Dryness of other areas may also be seen. Other prominent manifestations include joint pains similar to that in rheumatoid arthritis. The exact cause of Sjögren’s syndrome is undetermined. However, it is caused by viral infections triggering autoimmunity or die to multiple other environmental and genetic factors contributing to the autoimmune issues. Diagnosis often begins with the patient consulting an ENT Specialist or an Ophthalmologist with complaints of dryness.”
He elaborated, “If these experts determine significant dryness, they refer the patient to a rheumatologist. The rheumatologist may then suggest an Anti-Nuclear-Antibody test. If the ANA test is positive and the dryness is clinically significant, the diagnosis of Sjogren’s syndrome is confirmed. The secretory glands in the oral cavity and eyes can get enlarged due to inflammation hence causing dryness which responds to steroids initially then the disease modifying drugs such as hydroxy chloroquine, methotrexate, and leflunomide are continued for further improvement. Sjögren’s Syndrome may also affect the lungs, nerves, other internal organs, brain, and spinal cord. Depending on the affected organ and the severity additional treatment with immunosuppressants or immunomodulators such as micophenolate mofetil, cyclophosphamide or rituximab injections may also be required.”
Dr Supriya Sriganesh, Executive Director and Phaco and Refractive Consultant at Nethradhama Super Speciality Eye Hospital in Bangalore, opined, “Sjögren’s Syndrome presents a spectrum of potential ocular complications, including uveitis, scleritis, retinal vasculitis, and optic neuritis, underscoring the critical need for comprehensive care. The diagnostic process hinges on evaluating the functionality of salivary and tear glands, coupled with an assessment for autoimmune evidence. The condition profoundly affects the mucous membranes and moisture-secreting glands in the eyes and mouth, culminating in reduced tears and saliva. The treatment approach for Sjögren’s Syndrome centers on symptom alleviation, proactive management, and complication prevention. Dry eyes, a prevalent outcome, stem from an aqueous tear-deficient state, diminishing tear production and giving rise to vision problems, eye pain, and redness.”
She added, “In the diagnostic phase, healthcare professionals rely on a thorough examination of medical history and eye tests. The Schirmer’s test, a crucial tool, aids ophthalmologists in quantifying moisture production, providing insights into the severity of dryness. Treatment plans encompass a multifaceted strategy, combining self-management approaches with over-the-counter medications. Symptomatic relief for dry eyes involves the use of eye drops, artificial tears, prescription ointments, and punctal plugs, creating a protective layer of tears over the eyes. The care model at our hospital emphasizes on the integration of these diagnostic and treatment modalities into a comprehensive plan, promoting the well-being of individuals grappling with Sjögren’s Syndrome and ensuring a nuanced approach to their visual health.”