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Rituximab is a very promising drug for the treatment of severe Sjogren's syndrome and may have a role in modifying the disease process based on its effective treatment for systemic lupus erythematosus (SLE) which also is characterized by significant B-cell hyperactivity.

Various studies of patients with Sjogren's syndrome who were treated with rituximab showed benefit for:

• Improvement of sicca syndrome
• Improvement in salivary gland production
• Improvement in extraglandular manifestations such as mixed cryoglobulinemia, refractory pulmonary disease, and peripheral neuropathy
• Severe arthritis
• B-cell lymphoma - small studies have reported complete remission in some patients with MALT lymphoma.

Adverse effects of rituximab include:

• Infusion reaction (up to 35% of cases)
• Neutropenia (abnormally low levels of neutrophils in the blood)
• Serum sickness (severe reaction due to the introduction of a foreign substance)

Rituximab is typically administered with corticosteroids and not as a monotherapy. In 2006, the FDA issued a warning regarding fatal progressive multifocal leukoencephalopathy in patients with SLE taking rituximab. This serious side effect has not been seen in patients with Sjogren's syndrome but clearly, patients on rituximab should be carefully monitored for the development of any neurological symptoms.

Eprazumab is another B-cell target therapy which was developed to treat non-Hodgkins lymphoma and has shown promising results for treatment with SLE. Small studies involving patients with Sjogren's syndrome are inconclusive but investigation is continuing on this promising medication.

For further information, please click on the following link: http://www.medifocus.com/abstracts.php?gid=RH001&ID=17586555

Management of Musculoskeletal Symptoms

Muscle and joint pain associated with Sjogren's syndrome can usually be effectively treated with:

• Analgesics such as aspirin or non-steroidal anti-inflammatory drugs (e.g., Motrin, Advil) are the first-line of treatment for musculoskeletal symptoms.

• Low-dose corticosteroid therapy (e.g., prednisolone) is usually reserved for patients suffering from:

  • severe joint pain or arthritis
  • cutaneous symptoms
  • severe oral and ocular sicca symptoms
  • myositis (inflammation of muscle tissue) and neuritis (nerve inflammation)

• Hydroxychloroquine (Plaquenil) -Short term studies noted improvement in musculoskeletal symptoms such as arthralgia, myalgia, malaise, or fatigue; as well as in some immune markers such as:

  • ANA - antinuclear antibody
  • rheumatic factor
  • erythrocyte sedimentation rate

Management of Fatigue

Patients with significant fatigue should also be evaluated and treated for other conditions such as:

• Depression
• Hypothyroidism
• Fibromyalgia
• Lymphoma

Exercise may be beneficial for some Sjogren's syndrome patients suffering from fatigue. Patients should consult their health care provider to determine what an appropriate level of exercise would be. A small study showed an improvement in fatigue after treatment with etanercept but there have not been any large-scale clinical trials which confirmed this finding.

Management of Vasculitis

Management of vasculitis may include:

• Corticosteroid creams
• Intravenous cyclophosphamide and high dose corticosteroids
• Plasma exchange (plasmapheresis) for severe complications of cryoglobulinemia vasculitis
• Intravenous immunoglobulins (IVIG)
• Rituximab

Raynaud's phenomenon may be treated with calcium-channel blockers or ACE (angiotensin-converting-enzyme) inhibitors. In addition, patients should try to avoid situations of physical or emotional stress.

Management of Pulmonary Symptoms