Successful therapy with intravenous immunoglobulin in the management of polymyositis.

Polymyositis is an inflammation of muscle tissue of unknown etiology. It is characterized by symmetric, mainly proximal muscle weakness, muscle fiber damage proved on biopsy, increased enzymes and myoglobin, and has corresponding electromyography findings. Other systems such as joints, lungs, heart, and gastrointestinal system are involved. Lung involvement is rather common. The most frequent symptom represents shortness of breath caused by muscle weakness. We report a case of a 66 year old woman with primary idiopathic polymyositis. The clinical state of the patient was complicated by progressive muscle weakness, dysphagia, and respiratory failure. Due to the ineffectiveness of the treatment with corticsteroids and cyclophosphamide, treatment with high doses of immunoglobulins was started. A total of 100 g of i.v. immunoglobulin therapy was administered beginning on the 13th day after hospital admission. The state of the patient progressively improved and after 7 weeks of treatment in a significantly improved state the patient was transferred to a Rehabilitation Unit. We therefore conclude that IVIg therapy may be an effective therapeutic approach for the treatment of acute complications of polymyositis, especially in cases in whom other therapeutic strategies are ineffective or harmful (Ref. 10). Full Text (Free, PDF) www.bmj.sk.

[Prolactin--a marker of the activity of connective tissue systemic disease?]. / Prolaktin--marker aktivity systémových ochorení spojiva?

The ethiopathogenesis of auto-immune diseases has not yet been fully explored. Auto-immune diseases develop in individuals with a genetic pre-disposition. Among the endogenous factors, also sexual hormones play a role, especially prolactin. The objective of the study was to use basal exam and TRH test to determine stimulated prolactin values in patients with a systemic connective tissue disease: systemic lupus erythematosus (SLE), systemic sclerosis, Sjögren's syndrome, dermatomyositis. PATIENT GROUP AND METHODS: The authors examined 43 patients with systemic connective tissue diseases: 20 patients with SLE, 7 patients with Sjögren's syndrome, 7 patients with systemic sclerosis and 9 patients with dermatomyositis. The mean age of the patients was 39.87 years. The control group consisted of 30 volunteers with a mean age of 35.17 years. OUTCOME: Prolactin level in the patients with systemic connective tissue diseases was 14.629 ng/ml, which is more than in the control group. A statistically significant increase in prolactin level was recorded 20 minutes after i.v. stimulation as compared with the control group (30.982 ng/ml). This statistically significant difference was still present 60 minutes after the TRH administration, with 16.43 ng/ml in the control group, and 36.890 ng/ml in the systemic connective tissue disorder patient group. The differences between prolactin levels for the different systemic connective tissue diseases were not statistically significant. A positive correlation in the patient group was found between the stimulated prolactin values and the FW value. On the whole, however, there was no clear correlation found between laboratory activity and prolactin levels. CONCLUSION: The authors found out that post-stimulation prolactin levels were statistically significantly higher in patients with systemic connective tissue disease as compared with the control group. Correlation analyses did not show a clear link between inflammatory activity and prolactin levels, and therefore the authors assume that medium increased values of prolactin are rather a risk factor for the disease than an inflammation marker in patients with a systemic connective tissue disease.

Efficacy of intravenous immunoglobulin treatment in lupus erythematosus chorea.

Chorea is a rare complication of systemic lupus erythematosus (SLE) and is strongly related to the presence of antiphospholipid antibodies. Various infections may also be triggering factors in the development of choreiform movements. Additionally, Salmonella infection is the most common opportunistic bacterial infection in SLE patients. We report a case of a 33-year-old woman with SLE who developed lupus erythematosus-associated chorea with multiple involuntary movements and cognitive disturbances. Because the methylprednisolone therapy administered appeared to lead to Salmonella enteritidis infection, intravenous immunoglobulin (IVIg) in a total dose 100 g was administered after which a remarkable improvement of the abnormal movements and cognitive function was noted. Within 7 days, the patient had returned to normal. We therefore conclude that IVIg therapy may be an effective therapeutic approach for the treatment of the acute cerebral complications of SLE, especially in cases in whom other therapeutic strategies are ineffective or harmful.