19 patients with mixed connective tissue disease (MCTD) were studied for their bone and articular manifestations by synovial scintigram, bone scintigram and bone X ray pohotogram. All of our 19 patients with MCTD had polyarthritis, Raynaud's phenomenon and swollen hands, and 11 of them had mild myositis. The sera from all patients were tested for the presence of antibody to RNP by ouchterlony technique. All of them were positive. Rheumatoid factor were found in 15 patients (78.9%). But none of them had rheumatoid nodules and articular deformities like swan nek deformity or boutonniére deformity. Abnormal hot area on synovial scintigram was present in 14 (73.7%). Location and duration of this abnormal hot area were nearly correlated to clinical arthritis. Abnormal hot area on bone scintigram was present in 4 (28.6%). It was not clear whether the location of this abnormal hot area was correlated to the location of radiographic abnormalities. Bone and articular radiographs showed abnormalities in 8 (42.1%). Osteoporosis were found in 6, erosions in 5, narrowing of joint spaces in 4, bone resorption of styloid process in 2, bone resorption of terminal phalanx in 1, juxtaarticular linear calcification in 1.
In conclusion the articular abnormalities of MCTD were relatively limited, with involvement primarily of the hands and wrists.

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Polymyalgia rheumatica (PMR) is a clinical entity characteristic with pains and morning stiffness of neck-shoulder-gurdle muscles. Although the prevalence of PMR has been thought to be very low in Japan to date, we experienced 13 patients these three years. There is necessity for us to clarify the characteristics of Japanese patients with PMR. These 13 patients were primarily diagnosed based on the criteria from Bird et al. and from Healey, and compared their clinical features and laboratory findings with rheumatoid arthritis (RA) and with polymyositis/dermatomyositis (PM/DM). We now propose preliminary diagnostic criteria for PMR as follow: 1) Older than 50 years old. 2) Remarkable muscle stiffness in the morning on neck, shoulders and/or gurdles. 3) Tenderness of proximal muscles without muscle atrophy or the elevation of myogenic enzymes. 4) Sudden onset. 5) Fever at onset. 6) No swellings, pains nor stiffness of hands, feet and fingers. 7) Corrected ESR more than 70 mm/hr or CRP titer higher than 3 positives. 8) Negative rheumatoid factor, antinuclear antibodies nor other autoantibodies. 9) More than 0.8 g/dl of serum α₂ globulin and/or more than 40 of CH₅₀. Definite diagnosis of PMR can be made with more than 7 fullfill ed criteria. PM/DM, RA, osteoarthropathy, cervical syndrome and adult-onset Still's disease should be excluded.
Significant increases of HLA-A11, A26 and Cw3 were demonstrated in the patients with PMR comparing with those in healthy Japanese population, however no association with HLA-DR nor DQ system was proved.

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We used an immunofluorescence method to study the distribution of lactoferrin in the colonic mucosa in eight ulcerative colitis patients. Peroxidase staining was also examined simultaneously, and the distribution of peroxidase positive cells was compared with that of lactoferrin positive cells.
Both lactoferrin positive cells and peroxidase positive cells were barely found in the endoscopically uninvolved mucosa and abundantly in the endoscopically involved mucosa, whereas they were moderately observed in the boundary zone which was considered as locating between the involved and the uninvolved portion. On the other hand, in four normal colons these cells were rarely found. It was also intensely indicated that peroxidase positive cells were to be neutrophils by using Giemsa staining.
Lactoferrin is a strong iron-binding glycoprotein that has been detected in secretions that bathe human mucosal tissues, in vitro studies has been showed that it has both bacteriostatic and bactericidal effects. Meanwhile, our previous studies have demonstrated that s-IgA system decreases in distribution in the colonic mucosa in ulcerative colitis. Accordingly, these results suggest that lactoferrin appears and/or increases in compensation for s-IgA system in the colonic mucosa in ulcerative clitis, and it has a significant role in the mucosal defense for bacterial infection.

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We studied the effect of steroid hormone (prednisolone), and mitomycin C on the growth of the B lymphocyte colonies.
Also we described an in vitro method for normal human peripheral B cell colony formation. After one day preincubation with 3)μg of PHA-P, irradiated T lymphocytes and a non-T cell population, these lymphocytes were cultured in methylcellulose gel with 30μg of PHA-P. Colonies more than 50 cells were counted under a dissecting microscope.
A clear relationship between the number of seeded cells and the growth of colonies was seen in methylcellulose gel. 570±333 colonies per 5.0×10⁵ seeded cells were detected at 4 days of culture. Cells in colonies had surface immunoglobulin as detected using FITClabeled anti-human F (ab)₂ rabbit serum by fluorescent microscope, and Giemsa staining revealed differentiation to plasma cells and lymphocytes but no E rosette-forming ability.
Then the effect of steroid hormone (prednisolone), and mitomycin C on the growth of B lymphocyte colonies was studied.
When these drugs were added for 60 min. before the suspention culture, the inhibition effect of the colony growth was observed by prednisolone but no mitomycin C. When these drugs were added in the semi-solid culture, the inhibition effect of the colony growth was observed with dose dependence.
These results suggest that the human B lymphocyte colony forming cells may be noncycling but sensitive to prednisolone.

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Clinical studies and typing for HLA-A, HLA-B, HLA-C and HLA-DR loci on 13 family cases with familial Sjögren's syndrome in Japan including three family cases which have experienced in our institute were performed.
In our cases, all patients had sicca syndrome alone without other connective tissue diseases. Four of the six patients had multiple involvements of organs such as renal tubular acidosis, chronic thyroiditis and partial hypopituitarism. Five of the six patients were subclinical Sjögren's syndrome. The patients with familial Sjögren's syndrome had same HLA antigens each other, but no HLA antigens specific for Sjögren's syndrome were found out in all patients.
Also, in 13 family cases reported in Japan including the present cases, common HLA antigens specific for Sjögren's syndrome were not observed.
From these results, it was concluded that the pathogenesis of Sjögren's syndrome is heterogeneous.
Also we stressed that further collections of cases with familial Sjögren's syndrome of both clinical and subclinical type are necessary to make clear the factors which play a role on the development of Sjögren's syndrome.

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Reiter's syndrome is a syndrome which has a classical triad of polyarthritis, conjunctivitis, and non-gonoccal urethritis, and is closely associated with histocompatibility antigen HLA-B27. In this paper we report a typical case of this syndrome with a review of fifty cases collected from Japanese literature.
A 38 year-old male admitted our hospital with chief complaints of joint pain, pyuria and photophobia. He had had several episodes of urethritis and iritis. On admission he had polyarthritis, conjunctivitis, iritis, urethritis and balanitis circinatum. Labolatory data included accelerated erythrocyte sedimentation rate, 3+ C-reactive protein, and the presence of HLA B-27. Sacro-iliac joint was irregular and narrow by X-ray. The diagnosis of Reiter's syndrome was made. Non-steroidal anti-inflammatory drugs were given for two months, but without effect. Within a few days of 30 mg prednisolone daily, his complaints disappeared and within one week inflammatory signs also disappeared.
As far as we could identify, there are 51 cases of Reiter's syndrome in Japanese medical literature. Among them thirty-three cases had a classical triad of the syndrome, and 18 cases had also cutaneous lesions. HLA was examined in 11 cases and 7 of them demonstrated B-27. Over 80% of the patients steoidal therapy was effective, an observation different from reported cases in English literature. Elevated serum levels of GOT and GPT are reported in five casesincluding our own case, a finding which is also not common in English literature. HLA B-27 is quite rare (0.5%) among Japanese and this may partly account for the lower incidence of this syndrome in Japan.

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A 30-year old woman was admitted to our hospital because of fever and subcutaneous nodules. Histological examination of skin biopsy specimen disclosed diffuse panniculitis with infiltration of phagocyting histiocytes and lymphocytes. At this monent, the diagnosis of Weber-Christian disease was made, and treatment with prednisolone resulted in symptomatic relief. One month later, fever and subcutaneous nodules recurred. Labolatory examination revealed pancytopenia, coagulation abnormalities, liver dysfunction and hyperlipidemia. Despite the treatment with high dose corticosteroid, cyclophoshamide, colchicine and plasmapheresis, her condition gradually deteriorated, and finally, severe bleeding tendency such as gastrointestinal bleeding and hematuria ocurred. After 11 months, she died of panperitonitis and meningitis, which complicated after the surgical treatment of colon perforation. We considered this case as cytophagic histiocytic panniculitis rather than Weber-Christian disease. Cytophagic histiocytic panniculitis is a variant of Weber-Christian disease, originally described by Winkelmann, R. K. and Bowie, W. in 1980. Main features of this disease include fever, panniculitis, hepatosplenomegaly, pancytopenia and bleeding tendency. Pathologically, infiltration of mature histiocytes to skin, liver, spleen, bone marrow or lymph nodes with hemophagocytosis is the characteristic finding. This entity might be included in reactive histiocytosis, because of its clinical similarlity and pathological findings. Here, we report a case of cytophagic histiocytic panniculitis, and discuss the pathogenesis and treatment of this rare disease.

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