Sjögren’s Syndrome is a chronic autoimmune disorder where the immune system mistakenly attacks moisture-producing glands, primarily the lacrimal and salivary glands. This leads to glandular destruction and the characteristic symptoms of severe dryness in the eyes and mouth. Because the inflammatory process is systemic, the disease can extend beyond these glands, potentially affecting multiple organ systems, including the joints, skin, nerves, and internal organs. Effective management requires specialized, coordinated care that addresses both local dryness and underlying systemic inflammation.
The Primary Physician Leading Treatment
The rheumatologist functions as the central specialist in managing Sjögren’s Syndrome due to their expertise in systemic autoimmune diseases. They confirm the diagnosis using clinical presentation and objective tests, such as specialized blood work detecting autoantibodies like anti-Ro/SSA and anti-La/SSB. Diagnosis may also involve a minor salivary gland biopsy, typically taken from the inner lip, to look for characteristic focal lymphocytic infiltration.
Once diagnosed, the rheumatologist focuses on controlling systemic manifestations, managing symptoms like chronic fatigue and inflammatory joint pain. Treatment often begins with disease-modifying anti-rheumatic drugs (DMARDs), such as hydroxychloroquine, to modulate the immune response and reduce inflammation. For severe systemic involvement, the rheumatologist may prescribe immunosuppressive agents or biologic therapies, such as rituximab, to target specific immune cells and prevent organ damage.
Managing Dryness and Oral Health
The hallmark symptoms of dryness, known as sicca, require dedicated care from specialists focused on the eyes and mouth. An ophthalmologist treats dry eye (keratoconjunctivitis sicca), which ranges from mild irritation to vision-threatening corneal damage. Treatment starts with preservative-free artificial tears and lubricants. For persistent inflammation, the ophthalmologist may prescribe topical immunomodulatory drops, such as cyclosporine or lifitegrast, to suppress the localized autoimmune attack. In severe tear deficiency, punctal plugs or cautery can be used to block tear drainage ducts, preserving natural tears.
Dental care is equally important, as the loss of saliva (xerostomia) drastically increases the risk of oral complications. The dentist or oral medicine specialist implements aggressive preventive strategies to combat rapid tooth decay and gum disease. This includes prescribing high-potency fluoride toothpaste and sometimes custom fluoride trays for intensive remineralization therapy. To stimulate residual salivary gland function, the dentist may recommend cholinergic agonists like pilocarpine or cevimeline. They also monitor for and treat oral candidiasis, a fungal infection that thrives in a dry environment, often using antifungal troches.
Addressing Internal Organ Complications
When Sjögren’s Syndrome affects major organs beyond the glands and joints, consultation with other specialists is necessary.
Pulmonologist
A pulmonologist is consulted if the disease causes inflammation and scarring in the lungs, known as interstitial lung disease (ILD). This complication can lead to a persistent dry cough and shortness of breath, requiring careful monitoring.
Nephrologist
A nephrologist is needed if the kidneys become involved, often manifesting as distal renal tubular acidosis (dRTA). This condition impairs the kidney’s ability to excrete acid, which can result in severe electrolyte imbalances and the development of kidney stones.
Neurologist
Neurological manifestations require the expertise of a neurologist. The most frequent issue is peripheral neuropathy, such as sensory polyneuropathy or small fiber neuropathy, causing burning or numbness in the extremities. The disease can also affect the central nervous system, leading to complications like myelitis or optic neuritis.
Dermatologist
A dermatologist manages skin involvement, such as cutaneous vasculitis, which appears as palpable purpura, usually on the lower legs. This skin manifestation often signals widespread systemic disease activity, requiring close collaboration with the rheumatologist for systemic treatment.
Coordinating the Multidisciplinary Team
The Primary Care Provider (PCP) acts as the central coordinator and general health manager, ensuring all specialized care is cohesive. Since patients see many different specialists, the PCP maintains an overview of the treatment plan and facilitates communication between providers. The PCP also plays a role in preventative health and screening for common comorbidities.
Patients with Sjögren’s Syndrome have an increased prevalence of conditions like autoimmune thyroid disease, hypertension, and osteoporosis. The PCP manages these general health issues and conducts routine screenings to detect them early. By integrating complex specialty care with routine medical management, the PCP ensures the patient’s health is addressed holistically, preventing fragmented care and optimizing long-term outcomes.