NEW YORK (Reuters Health) Jun 30 - Intravenous immunoglobulin (IVIg) may offer some relief for patients with sensorimotor neuropathy or nonataxic sensory neuropathy associated with primary Sjogren's syndrome, researchers from France report.

IVIg is an established therapy for chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and Guillain-Barre syndrome, and several case reports have suggested that IVIg may have value in Sjogren's syndrome-associated peripheral neuropathy.

Dr. Xavier Mariette from Hopital de Bictre, Le Kremlin Bictre and colleagues in Club Rhumatismes et Inflammation assessed the effects and tolerability of IVIg treatment in a small retrospective study of 19 Sjogren's syndrome patients (mean age, 60 years) with neuropathy (median duration of neuropathy, 9 years).

Patients received intravenous IVIg (2 g/kg) for 5 days a month (10 patients) or 2 days a month (9 patients) for a median 7 months. Response was assessed using the disability Modified Rankin Scale and a global evaluation by the practitioner.

Results, with a median follow-up of 27 months, were published online May 16th in Arthritis Care & Research.

All five patients with sensorimotor neuropathy, all four with nonataxic sensory neuropathy, and the sole patient with conduction block improved or stabilized on IVIg therapy. In contrast, only two of nine patients with ataxic neuropathy improved, and four worsened. Disease remained stable in the other three patients.

The nine patients who had dramatic improvements showed responses after only two infusions.

"Because of the gravity of peripheral neuropathy and the absence of efficient treatment, even the stabilization of clinical symptoms would be a valuable goal since the natural course of this disease leads to a spontaneous and continuous aggravation," the researchers note.

After four to 12 months of treatment, five patients were able to have their IVIg infusions spaced every two or three months.

Ten of 13 patients who required corticosteroids were able to reduce their prednisone dosage from an average of 15 mg daily before IVIg to 10 mg after IVIg.

There were no severe side effects associated with IVIg treatment, and only one patient stopped treatment after one infusion because of nausea and lack of initial efficacy.

"Taking into account our results, from a practical point of view, symptomatic treatment should be tested in patients with Sjogren's syndrome without necrotizing vasculitis related neuropathy," the investigators conclude. "In this case, IVIg may be tried for at least two courses (and thus evaluation at the time of the 3rd course)."

"Further studies are necessary to investigate the optimal number of IVIg courses necessary to assess definitely the efficacy or the failure of the treatment, and to know how long the treatment should be continued," they add. "These studies should probably focus on one type of neuropathy (probably the sensorimotor or nonataxic sensory polyneuropathy)."

Sjögren's syndrome (SS) is a chronic inflammatory or rheumatologic disorder. Patients have decreased gland function, especially of the lacrimal, or tearing, glands, leading to dry eye. They may also have problems with the salivary glands, leading to dry mouth. This combination of dry eyes and dry mouth is called the sicca complex. It is the only manifestation of the disease in one-third of SS patients.

Patients with the dry eye syndrome may complain of a gritty, sandy feeling in the eye instead of dryness. They can also have corneal ulcerations and other eye diseases caused by the dryness. Dry mouth can cause candidiasis, or fungal infections of the mouth. Dry mouth can also lead to severe inflammation of the mouth and difficulty with gum health and difficulty supporting teeth (gingivitis). Dental caries are a significant problem.

Other common problems associated with SS are enlargement of salivary and lacrimal glands. Dryness of the upper airway tract is a reported problem. These patients often have sinus and throat problems, which can lead to a cough and in severe cases, pneumonia. Some female patients will complain of severe vaginal dryness.

Fatigue is common in Sjögren's syndrome. It may be because of the disease itself or to difficulties staying asleep, which might occur if one drinks a lot of water to treat dry mouth and then needs to urinate frequently at night.

SS can occur by itself or with other rheumatoid diseases. When occurring with other diseases, it is most commonly seen with rheumatoid arthritis. Fibromyalgia, a disease involving nonspecific muscle aches, is also common.

Non-Hodgkin lymphoma, a malignancy of the immune system, occurs in 2.5 percent of SS patients followed for 10 years. Many patients who succumb to the disease actually die of infections cause by the immune dysfunction that characterizes this disease.

The diagnosis of SS is usually confirmed through biopsy of a salivary gland, which is a relatively simple surgery, or through a blood test looking for specific antibodies.

The treatment of this disease is largely keeping affected areas hydrated. There are some medications beyond artificial tears for dry eye. There are also some treatments for dry mouth. Some patients find room humidifiers comforting.

Patients with this syndrome do much better if they understand the disease. For example, some common over-the-counter cold medicines need to be avoided as they can worsen symptoms. I have found the Sjögren's Syndrome Foundation, Inc., and the Arthritis Foundation to be very helpful in explaining the syndrome. The National Institute of Neurological Disorders and Stroke of the National Institutes of Health is the largest sponsor of Sjögren's research. It also has a listing of clinical trials that are still being enrolled.

The factors that cause Sjorgren’s Syndrome are still mostly unknown. Researchers around the country are continually studying those who suffer from the illness in order to learn more about its cause. Currently there is no cure for Sjorgren’s Syndrome and with good management of symptoms patients can live a long, normal life.

Sjorgren’s Syndrome is an autoimmune disorder where the infection fighting white blood cells attack glands throughout the body focusing on the ones that produce moisture and lubrication. Symptoms of Sjorgren’s can vary widely from person to person and it may take years to get an accurate diagnosis.

Nine out of ten patients with Sjorgren’s are women, and the majority of those are over the age of 40. However Sjorgren’s does not discriminate and has been found to effect men and children as well. Researchers are looking for links between genetics, hormones and other connective tissue diseases hoping to find a common factor that may contribute to Sjorgren’s.

For those who suffer from Sjorgren’s some symptoms are common. Dryness of the mouth and eyes affects nearly all sufferers of Sjorgren’s Syndrome. Being without these basic bodily functions can be very disruptive to normal daily activities. Learning to manage the symptoms is the best defense for Sjorgren’s until a cure is found to cure sjogren’s syndrome. Without proper moisture your eyes may be more likely to become infected, or you may have difficulty seeing clearly. Without saliva your mouth’s defenses against bacteria are weakened. Patients are more likely to suffer from tooth decay, or cavities and may have a decreased ability to taste what they eat. Prescription eye drops are treatments for sjogren’s syndrome may help with tear production and synthetic liquids for the mouth can help replace moisture that saliva normally would.

Whereas symptoms can be handling with prescription medications, natural alternative for sjogren’s syndrome or some purchased over the counter. The prescription eye drops may assist with tear production and synthetic liquids for the mouth can help restore moisture that saliva normally would. Considering the internal organs are involving, your doctor may prescribe immune suppressive medications-signifying that they prevent the immune system to keep the white blood cells from assaulting which may help slowing the symptoms.

For healthcare professionals diagnosing primary Sjogren's Syndrome (pSS, an autoimmune disorder in which immune cells attack and destroy moisture-producing glands), the incidence of blood based deficiencies is the strongest predictor of a poor outcome in patients according to the results of a study presented at EULAR 2010, the Annual Congress of the European League Against Rheumatism in Rome, Italy. The study also showed that liver and lung involvement and non-Hodgkin lymphoma (NHL) development were also related to an increased mortality in pSS patients.

Results of a Spanish study have shown that patients who present with concurrent anemia (a reduced number of red blood cells), lymphocytopenia (a reduced number of white blood cells), or hypocomplementemia (where components of blood are lacking or reduced) were the most likely to develop poor outcomes, such as lymphoma. The existence of pulmonary (lung fibrosis, brochiectasis) and hepatic (biliary cirrhosis, autoimmune hepatitis) involvement were also shown to be independent risk factors related to mortality.

"Whilst pSS is often characterised by changes in exocrine function, the results of our study have determined the profile of some of the non-exocrine signs of the disease, including the pulmonary, haematological and hepatic manifestations," said Professor Roser Solans-Laqué, Internal Medicine, Vall D'Hebron University Hospital, Barcelona, Spain. "We hope that the results of our study will enable a better understanding of the factors that impact prognosis with the aim of monitoring for and managing these appropriately in the future."

Two hundred and forty-four patients (females n=235 males n=9) with pSS (primary SS occurs as a disorder on its own, with no known association with another connective tissue disease or rheumatic condition) registered at the Vall D'Hebron University Hospital, Barcelona were included in this single centre study. Mean age was 58 years, and clinical follow up ranged from nine months to 20 years (mean 8.6 years). Statistical analyses including multiple logistic regression were undertaken to determine associations between disease manifestations and patient outcome. At 20 year follow up, 11 patients (4.5%) had developed non-Hodgkins lymphoma, 22 (9%) patients had developed other malignancies (including lung, colon, breast and gynaecological neoplasms), and 18 (7.4%) of patients had died. Only an excess mortality due to lymphoproliferative malignancies was found in patients with pSS.