Eizo Saito

Polymyositis and dermatomyositis associated with malignancy: a 30‐year retrospective study

Background Polymyositis and dermatomyositis in association with malignancy are paraneoplastic syndromes, but the incidence, treatment and factors that predict associated cancer and its prognosis all remain unclear.

Effects of intravenous immunoglobulin therapy in Japanese patients with polymyositis and dermatomyositis resistant to corticosteroids: a randomized double-blind placebo-controlled trial

High-dose intravenous immunoglobulin (IVIG) therapy has been effective in treating various autoimmune and systemic inflammatory diseases. Here, we assessed the efficacy and safety of IVIG therapy with polyethylene glycol-treated human IgG (drug code GB-0998) for patients with corticosteroid-refractory polymyositis (PM) and dermatomyositis (DM) by means of a randomized, double-blind, placebo-controlled study. We randomly assigned 26 subjects (16 PM and 10 DM) to receive either GB-0998 or placebo. Intragroup comparison in the GB-0998 group showed statistically significant improvements due to GB-0998 administration in the primary endpoint (manual muscle test score) and secondary endpoints (serum creatine kinase level and activities of daily living score). However, significant improvements were also found in the placebo group, and comparison of the GB-0998 group with the placebo group did not show any significant difference between the groups. We discuss possible reasons for the absence of a clear intergroup difference in efficacy. Nineteen adverse drug reactions were observed in 11 of 26 subjects (42.3%), of which 2 events (decreased muscle strength and increased serum creatine kinase) were assessed as serious; however, they are previously known events. These results indicate that GB-0998 can be safely used with the same precautions as other current IVIG therapy.

Prospective study of high-dose intravenous immunoglobulin for the treatment of steroid-resistant polymyositis and dermatomyositis

Abstract High-dose intravenous immunoglobulin (IVIG) therapy has been effective in treating many autoimmune and systemic inflammatory diseases. In the present prospective study, we evaluated the efficacy of IVIG for patients with polymyositis (PM) and dermatomyositis (DM) refractory to treatment with high-dose corticosteroids. PM/DM was defined as steroid-resistant when the muscle strength of a patient did not improve despite the administration of more than 50 mg prednisolone per day for more than 4 weeks. A total of 12 patients with biopsy-proven, steroid-resistant PM/DM received one infusion of polyethylene glycol-treated human IgG at a dose of 0.4 g per kg per day for five successive days. Three of the patients received a second infusion. All patients were followed for up to 3 months after the infusion. Finally, 8 patients (6 PM and 2 DM; 5 men and 3 women) aged 29–67 years (mean 48 years) were analyzed. Their clinical response was assessed by changes in (a) subjective signs, i.e., fatigue (visual analog scale, VAS), muscle pain (VAS), activities of daily living (ADL), (b) objective signs, i.e., manual muscle strength (MMT) and serum level of creatine kinase (CK). At 12 weeks after the infusion, the patients showed significant improvement in their scores of muscle strength (from a mean of 67.0 to 81.0) and their ADL scores (from a mean of 27.1 to 39.1). The mean serum CK level decreased significantly from 1287.4 to 612.6 IU/l. In addition, the mean VAS of fatigue decreased significantly from 5.5 to 1.3 cm. The physicians’ assessment showed that 87.5% of patients had improved. The average reduced dose of prednisolone was 47.1 mg/day at 12 weeks after infusion in 7 patients who exhibited improvement. Adverse effects, i.e., asymptomatic myocardial infarction and increased blood urea nitrogen (BUN), were noted with two of the 15 infusion (13%). Overall, IVIG was found to be safe and effective for refractory PM and DM.

What is the factor that most influences QOL among rheumatoid arthritis patients

Abstract We attempted to elucidate the factors which affect the quality of life (QOL) among patients with rheumatoid arthritis (RA). Ninety-five patients who satisfied the American Rheumatism Association criteria for RA were asked to fill in a modified arthritis impact measurement scale, version 2 (AIMS2) and complete a Lorishs face scale (FS) test. The same questionnaire and FS test were completed by 75 healthy persons as controls. We used Lorishs FS for our assessment of QOL. The investigation was undertaken to analyze the relationship between FS and each item on the questionnaire. For average FS score, there was no significant difference between the RA group and the controls. However, RA group scores covered a wider range than those of the controls. From the correlation analysis, physical stress, pain factors, and some of the activities of daily living (ADL) factors showed a strong correlation with FS. ADL factors which strongly correlated with FS were those related to activities of the lower limbs. Other ADL factors were moderately correlated with FS. Socioeconomic factors were not significantly correlated with FS. There was no significant difference between the QOL of RA patients and that of healthy controls. The QOL was correlated with pain and stress factors rather than with ADL factors among patients with RA.

Thrombotic thrombocytopenic purpura in a patient with a diffuse form of systemic sclerosis

This paper presents a patient with a rapidly progressive diffuse form of systemic sclerosis, in whom a clinical course thrombotic thrombocytopenic purpura (TTP) also developed. In spite of treatment with plasmapheresis, predonisolone, aspirin and vincristine, she died of pulmonary bleeding. From the analysis of four other reported cases of scleroderma with TTP and this case, the outcome of TTP seems to be closely related to clinical manifestations. It is suggested that a diffuse form of scleroderma with interstitional manifestations would develop a worse outcome; on the other hand, a limited form without interstitional manifestations has a successful outcome.

Rapidly progressing neurological disturbance due to intraspinal calcification in a patient with systemic sclerosis

Abstract A 53-year-old woman with diffuse cutaneous systemic sclerosis (dsSSc) who developed muscle weakness in her lower extremities was admitted to our hospital. Computed tomography (CT) of her thoracic spine showed paraspinal and intraspinal calcifications producing severe spinal stenosis. After admission, her neurological symptoms, including muscle weakness and sensory disturbance, rapidly progressed and finally her lower extremities became completely paraplegic. After initiation of diltiazem and bucillamine, her neurological disturbance showed a marked improvement. A CT scan of the thoracic spine after medication showed dominant decrements in both intraspinal and paraspinal calcifications.

[Reevaluation of glucocorticoid therapy on polymyositis: dose-response relationship between glucocorticoid and long-term therapeutic effect].

多発性筋炎(PM)における糖質コルチコイド(GC)療法の治療効果を客観的に評価した成績は少なく,長期予後に対する効果は知られていない.そこで, PM 59例を対象に治療効果,副作用出現を用量反応性の面から検討した. GC大量投与例は短期,長期とも機能予後に対する治療効果が優れていた.しかし, GC薬投与に伴う重篤な副作用の出現率も高かった.一方,中等量以下で長期的な機能改善の得られる例も存在した. GC薬投与量と生命予後の間には明らかな関連は無かった.以上の成績から, GC薬はPMの長期的な機能予後を改善し得る事が判明したが,今後,症例による投与量の選択,内臓病変に対する治療法の確立が重要であると考えられた.

Large molecular weight ACTH immunoactivity in a patient with adrenoleukomyoloneuropathy

Very high ACTH immunoactivity was measured in a patient with adrenoleukomyeloneuropathy. The basal adrenocortical function was hyperactive, whereas the response to exogenous ACTH was depressed. Most of the ACTH immunoactivity was a large molecule (55,000 daltons), which weakly stimulated steroidogenesis of adrenocortical cells in vitro, but inhibited the binding of 125I-iodoACTH to the cells and suppressed ACTH-induced steroid production. Abnormalities in the pituitary-adrenal axis in adrenoleukomyeloneuropathy have been considered to be solely attributable to destruction of the adrenal cortex. In the current case, however, large molecular weight ACTH immunoactivity is present and might have some role in the adrenocortical dysfunction.