[in Japanese]

View full abstract

    Currently, measurement of anti-SS-A and anti-SS-B antibodies has been carried out for most diagnostic reagents based on the principle of enzyme-linked immunosorbent assay. The results are reported as numerical data. However, values are not standardized because every reagent manufacturer uses different evaluation methods. We clarified the relationship between the measured values of seven reagents made by six companies. We then observed whether the measured value of each reagent could be matched by establishing a fixed standard. Standardization of anti-SS-A/B antibody using enzyme immunoassay was attempted using pooled serum, a CDC reference, and clinical samples, but this was difficult. Since more than a 32 times or higher titer of anti-SS-A antibody on double immunodiffusion (DID) was extracted as a factor suggesting congenital heart block (CHB), the values measured using various reagents corresponding to a DID titer of 32 times were estimated, but some cases were judged as negative based on the estimated value. Moreover, since the objective of the clinical use of an enzyme immunoassay is mainly ‘qualitative judgment’, many companies have not reported measured values of the reagents as the original antibody titers (particularly, for high-titer cases). Considering this current situation, a proposal for the standards requiring confirmation using DID was prepared: Confir-mation using DID is necessary when the value is 100 EU or higher on measurement using Bio-Rad, 80 units or higher using INOVA, an index value of 100 or higher using Cosmic,300 U/ml or higher using TFB, 240 U/ml or higher using Phadia, an index value of 100 or higher using MBL MESACUP, and 500 U/ml or higher using MBL STACIA.

View full abstract

    Mizoribine (MZR) is a second-line immunosuppressive drug that has been used in Japan to prevent rejection of renal allografts, and also for treatment of rheumatoid arthritis, nephrotic syndrome, immunoglobulin A nephropathy and systemic lupus erythematosus. We report a 47-year-old woman who was diagnosed as having interstitial pneumonia and dermatomyositis. She was initially treated with corticosteroid and azathioprine, but during tapering of the corticosteroid, the interstitial pneumonia recurred. She was therefore treated with mizoribine and corticosteroid, and this led to complete resolution of the disease. In this case, mizoribine was used as a second-line treatment and steroid-tapering agent. Mizoribine appears to be a useful agent for treatment of chronic interstitial pneumonia in polymyositis/dermatomyositis, and the present case is the first to have been reported in which the drug has been used successfully for treatment of interstitial pneumonia with dermatomyositis.

View full abstract

    A 74-year-old man was admitted into our hospital for fever, dyspnea on exertion and right shoulder pain. While body CT scan and colonoscopy showed interstitial pneumonia and erosion of large intestinal mucosa, respectively, the diagnosis could not be defined without any other findings including negative results of both MPO-and PR3-ANCA. Since fever and elevated CRP were spontaneously improved, he was discharged. One month later, he suddenly had dysarthria and right hemiplegia. CT scan revealed bilateral putaminal hemorrhage. Cerebral hemorrhage, interstitial pneumonia, erosion of large intestinal mucosa, and elevated CRP suggested some kind of vasculitis. Therefore, we started the administration of prednisolone 1 mg/kg/day, while no criteria of vasculitis were fulfilled. Cyclophosphamide was not used due to possibility of accompanied diverticulitis of the colon. Four weeks later, he died of respiratory failure. In the autopsy, fresh and old necrotizing inflammation of the medium-sized muscular arteries of cerebral basal ganglia, mesenterium, and heart (coronary artery) was demonstrated, while crescentic glomerulonephritis or vasculitis was not observed in arterioles, capillaries, or venules. These findings resulted in the diagnosis of polyarteritis nodosa which had been difficult to diagnose clinically.

View full abstract

    Recently, it has come to be known that there are some patients who are suffering from arthralgia, Raynaud’s phenomenon, or with serum anti-nuclear antibodies suggesting connective tissue disease although their clinical findings do not fulfill the criteria of any specific connective tissue diseases. Such pathophysiology is referred to undifferentiated connective tissue disease (UCTD). The condition of patients with this pathophysiology is frequently not very serious, and disease activity is overcome with a relatively small amount of corticosteroids. Here we present a case where serum anti-DNA antibody was positive, and digital ulcers in the patient’s feet progressively developed to necrosis. Angiography and pathological examination suggested that she has a vasculitis different from thromboangiitis obliterans (TAO), arteriosclerosis obliterans (ASO) or panarteritis nodosa, but she was thought to have UCTD because of clinical and pathological findings. This case suggests that there some cases may be serious even in the case of UCTD, suggesting that early pathological examination is essential for diagnosis and treatment.

View full abstract

[in Japanese]

View full abstract

[in Japanese]

View full abstract

[in Japanese]

View full abstract

[in Japanese]

View full abstract

[in Japanese]

View full abstract

[in Japanese]

View full abstract