IVIG treatment appears to be safe, able to ease range of SSc symptoms
Given as add-on therapy for muscle inflammation, skin and digestive problems
Including intravenous immunoglobulin (IVIG) as part of routine care may help to ease muscle, skin, and digestive symptoms in people with systemic sclerosis (SSc), a Spanish study found.
The experimental treatment was seen to be generally safe, with reported side effects being mostly mild or moderate.
While these findings point to IVIG as a potential treatment option for scleroderma, “randomized clinical trials are required to assess the role of IVIG in the treatment of SSc manifestations,” its researchers wrote.
The study, “Immunoglobulins in systemic sclerosis management. A large multicenter experience,” was published in Autoimmunity Reviews.
Most scleroderma patients treated with IVIG in study had diffuse disease
A buildup of dense, scarred tissue in scleroderma causes the skin to harden and thicken. Sometimes, this also affects other organs in the body, leading to problems with muscles and the gastrointestinal (digestive) system.
SSc treatments are directed at easing inflammation and scarring or managing symptoms. However, because symptoms can be many, a combination of approaches often is needed.
“There is no disease-modifying therapy and treatment is based on the management of the clinical features and complications,” the researchers wrote.
Given as an infusion into a vein, IVIG is a pool of immunoglobulins (antibodies) collected from the blood of healthy donors. It is used to treat infections or immune diseases characterized by low blood antibody levels.
Some earlier work has suggested that IVIG may ease muscle and digestive symptoms in people with SSc and help to reduce the use of corticosteroids, which can have serious side effects with long or high dose use.
To better understand the safety and effectiveness of IVIG in treating scleroderma symptoms, the researchers looked at the medical records of 78 adults (64 women, 14 men), all with an SSc diagnosis and a mean age of 58.5, from 18 hospitals across Spain.
Most patients, 46 or 59%, had diffuse SSc, the disease’s most severe form, and 32 (41%) had limited SSc.
Half of these people also had another connective tissue disease. The most common was idiopathic inflammatory myopathy (41%), an inflammation of skeletal muscles — those used for movement — due to unknown reasons, and Sjögren’s syndrome (6%).
Patients given IVIG in monthly cycles in combination with standard care
IVIG was given most often to treat myositis, or inflammation of the muscles (49%), followed by gastrointestinal (31%) and skin symptoms (22%). In seven (9%) patients, IVIG was used to treat more than one symptom.
Patients were treated with IVIG at a median of five cycles each month. Most received IVIG in combination with corticosteroids (69%), immunosuppressants (67%), or biologics (18%). The most commonly used immunosuppressant was mycophenolate mofetil.
Muscle strength was evaluated before IVIG and three months after the last treatment cycle using a modified version of the Medical Research Council scale. This scale rates muscle strength from grade 5 (normal) to grade 0 (no visible muscle contraction, or tightening).
About 92% of treated patients had muscle strength grades of three to five, suggesting IVIG may be easing muscle symptoms.
The levels of creatinine kinase decreased significantly, by more than five times, falling from an average of 1,149 to 217 international units per milliliter of blood. Creatinine kinase is an enzyme mostly found in skeletal muscles, and high levels in blood can indicate muscle damage.
IVIG treatment also eased skin symptoms, as seen by a significant two-point decrease — from 15 to 13 points — in the average modified Rodnan skin score (MRSS), a measure of skin thickness.
A questionnaire, the University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA GIT 2.0), was used to assess quality of life related to gastrointestinal symptoms. Higher scores here indicate a poorer quality.
Before IVIG, patients had an average score of 1.06 points in this questionnaire. Mean scores decreased significantly to 0.78 after treatment, suggesting that IVIG also helps to ease gastrointestinal symptoms.
A total of 12 side effects were reported in 10 patients, the majority being mild or moderate in severity. Treatment was stopped in four patients, one due to a serious side effect.
“Our findings suggest that IVIG should be a treatment option in cases of myositis, [skin] and gastrointestinal involvement in patients with SSc,” the researchers concluded, adding the treatment “has a good safety profile.”
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