Taeko Tamura

Antineutrophil cytoplasmic autoantibodies in patients with systemic sclerosis

Perinuclear type of antineutrophil cytoplasmic antibodies (p‐ANCA) have been found in patients with periarteritis nodosa. Churg‐Strauss arteritis. or pauci‐immune idiopathic crescentic glomerulonephritis. Recently, the association of p‐ANCA and normotensive renal failure, in patients with systemic sclerosis (SSc), was reported. We have studied the incidence of p‐ANCA in patients with SSc and have investigated its relationship to clinical and laboratory findings. Sera from 77 patients with SSc were examined by the indirect immunofluorescence (IIF) test, employing an ethanol‐fixed human neutrophil and enzyme‐linked immunosorbent assay, using purified myeloperoxidase (MPO) as an antigen (MPO‐ELISA). The sera from seven patients (9‐1%) were p‐ANCA positive, by both IIF and MPO‐ELISA. One patient died from systemic necrotizing angiitis but the remaining six patients have shown no symptoms of systemic vasculitis or of renal involvement. There was a tendency for patients positive for p‐ANCA (anti‐MPO antibody) to also be positive for other autoantibodies. such as anti‐Sc1‐70 antibody, anti‐centromere antibody, anti‐microsome antibody, anti‐thyroglobulin antibody and rheumatoid factor. Although the incidence of p‐ANCA (anti‐MPO antibody) is low in patients with SSc, and its clinical significance in SSc needs further investigations, this could be a serological marker for certain symptoms in SSc.

Measurement of Skin Elastic Properties with a New Suction Device (II): Systemic Sclerosis

We previously reported the skin elastic properties of normal subjects as measured by a newly developed suction device (6). In the present report, we examined the skin elastic properties of 62 patients with systemic sclerosis (SSc), clinically diagnosed as limited or diffuse type. We measured the skin elastic properties on the forearm and chest and investigated correlations with disease type and clinical skin score. Patients with the diffuse type SSc showed significantly lower values than those with the limited type SSc or normal subjects in distention and retraction ability at the chest and forearm.

Lipodystrophia centrifugalis abdominalis infantilis Occurring in the Neck

We report a Japanese girl with atypical lipodystrophia centrifugalis abdominalis infantilis. The initial lesion developed on the neck as an area of erythema which showed centrifugal spread to the nape, submandibular area and upper breast and central fading to leave a residual depression and purplish brown pigmentation symmetrically. A central depression on the neck, nape, submandibular area and upper breast was surrounded by a distinctive erythematous, slightly elevated and indurated border. Histological examination of the erythematous border revealed inflammatory changes in the subcutaneous fat. Although this patient was affected in an unusual site, we concluded that she had lipodystrophia centrifugalis abdominalis infantilis, because of the overall features of the lesions.

Systemic sclerosis terminating as systemic necrotizing angiitis

We report a 57‐year‐old woman with systemic sclerosis (SSc) who died suddenly, following a haemoptysis. At post‐mortem, systemic necrotizing angiitis of small vessels was observed in several organs. Necrotizing angiitis has been reported as a rare complication of systemic sclerosis, and is usually lethal.

Image analysis of nailfold capillaries in patients with undifferentiated connective tissue syndromes.

Several studies have reported patients that show nailfold capillary abnormality without fulfilling any of the criteria for rheumatic diseases. Our objective was to define how many patients with undifferentiated connective tissue syndrome (UCTS) have nailfold capillary abnormalities and to determine the correlation between capillary abnormality and clinical findings. We analyzed videograph images of nailfold capillaries in 75 patients with UCTS, comparing them with 22 normal controls (NL) and 55 patients with systemic sclerosis (SS), using standardized canonical discriminant analysis. Sixty patients with UCTS showed the SS type pattern and 15, the NL type pattern. The SS type pattern in patients with UCTS significantly correlated with Raynauds phenomenon, telangiectasia, and anti‐nuclear antibody. The UCTS patients with nailfold capillary abnormalities correlated with symptoms of SS have a possibility of progressing to SS. The follow up study of these patients will show whether progression to SS occurs.

Activation of Fibroblasts Induced by Mononuclear Cells from Patients with Systemic Scleroderma and Inactivation by Mitogen Stimulation

Unstimulated mononuclear cell supernatants from patients with systemic scleroderma significantly augmented the DNA and collagen biosynthesis of 3T3 cells compared to normal mononuclear cells. This increase was suppressed by either a particular glycosaminoglycan, which from previous studies, has appeared to act as one of the tissue antigens in sclerotic skin, or concanavalin A stimulation.

Clinical Significance of Lymphocyte Responsiveness to Scleroderma-Inducing Glycosaminoglycan-Associated Antigen in Systemic Scleroderma

Twenty-nine patients with systemic scleroderma (SSD), whose lymphocytes significantly responded to a scleroderma-inducing glycosaminoglycan-associated antigen (stimulation index greater than or equal to 1.7), were separated into the two groups of marked and moderate responses, according to the mitogenic response of their peripheral lymphocytes. A marked response, with a [3H]-thymidine uptake of +3 SD over the mean of unstimulated SSD lymphocytes, was seen in severely affected patients with a high OKT4/OKT8 ratio. In contrast, the lymphocytes of patients with benign disease responded moderately to the antigen, showing lower thymidine uptake by both unstimulated and stimulated lymphocytes. This latter group had a high frequency of elevated anti-nRNP antibody.