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Severe pulmonary symptoms are rare in Sjogren's syndrome but, if they do develop, may be treated with corticosteroids. Pneumonia or bronchitis is typically treated with antibiotics. Some clinicians recommend immunization against pneumococcal infection. Use of a humidifier to keep the air moistened may also relieve some symptoms such as a dry, hacking cough.

Management of Renal Symptoms

Renal (kidney) involvement in patients with Sjogren's syndrome is typically subclinical. In severe cases, management may include:

• Corticosteroid therapy
• Pulse intravenous cyclophosphamide combined with prednisolone for glomerulonephritis
• Oral potassium and sodium bicarbonate for acidosis
• Plasmapheresis (exchange of plasma)

Management of Gastrointestinal and Hepatobilliary Symptoms

• Gastroesophageal reflux can usually be controlled with medications such as antacids, histamine-2 (H-2) blockers, or proton-pump inhibitors.
• Sjogren's patients who develop mild hepatitis may require no specific treatment, however, liver function tests should be performed to monitor the course of the hepatitis. Patients with persistently elevated liver function tests may be treated with corticosteroids and azathioprine.
• Some patients with Sjogren's syndrome develop a condition called primary biliary cirrhosis that can lead to cirrhosis (hardening) of the liver. Treatments for primary biliary cirrhosis may include vitamin and calcium supplementation and other symptomatic treatments. In cases where liver damage is extensive, liver transplantation may be necessary.

Management of Neurological Symptoms

Peripheral and sensory neuropathy typically do not respond well to treatment but they often stabilize over time. Some patients with severe sensory neuropathy reported benefit from infliximab which may indicate that TNF-alpha drugs play a role in patients with other severe extraglandular involvement. Some patients with peripheral or cranial neuropathy and Sjogren's syndrome report benefit from other medications such as:

• Corticosteroids
• Pulse intravenous cyclophosphamide combined with corticosteroids
• Azathioprine and methotrexate (if cyclophosphamide is not effective or not well tolerated)
• Low-dose antidepressants
• Anti-inflammatory agents
• Anticonvulsive agents (e.g., gabapentin)
• Plasmapheresis

Patients who fail to respond to more conservative measures should be thoroughly evaluated by a neurologist who can determine if there are other treatment options available.

Management of Gynecologic Symptoms

Vaginal lubricants can help make intercourse less painful and more comfortable. Vaginal estrogen creams may also be used to treat vaginal dryness by increasing capillary blood flow to the vaginal and vulvar area. Creams containing vitamin E or vitamin E oil are effective for lubricating the external vulvar surface and for relieving painful intercourse.

Examples of creams that may relieve vaginal dryness include:

• Replens (polycarbophil)
• Durex Sensilube

Both of these creams are able to cling to the vaginal surface and rehydrate the surface cells and are effective for up to 72 hours. Cortisone creams should be avoided.

Management of Hematologic Symptoms

Hematologic symptoms associated with Sjogren's syndrome are managed with medications including:

• Corticosteroids
• Immunosuppressants
• Azathioprine
• Cyclophosphamide
• Methotrexate
• Intravenous immunoglobulin (IVIG) combined with corticosteroids
• Plasmapheresis

Management of Lymphoproliferative Disorder

• Rituximab is a first line therapy for B-cell lymphomas. Most lymphomas in Sjogren's patients are treated with the same protocols as lymphoma in the general population.
• Epratuzumab is under investigation for treatment of B-cell lymphoma