To clarify the clinical features of acute interstitial pneumonia in dermatomyositis and polymyositis (DM/PM) patients with a poor prognosis, we examined retrospectively 18 patients of the acute interstitial pneumonia out of 71 patients of DM/PM. Ten out of 18 were patients with a poor prognosis, died of acute respiratory failure and the other 8 patients had a good prognosis. One out of 5 autopsied lungs in poor prognosis cases was cytomegalovirus pneumonia while the others were UIP. All the patients of poor prognosis had low CPK and low aldolase level, and showed less inflammatory signs than those with a good prognosis when their interstitial pneumonia occurred. All the male patients, 4 out of 18, were in a poor prognosis group. Typical skin rash such as heliotrope and Gottron's sign was more common in a poor prognosis group. Seven patients with poor prognosis showed interstitial pneumonia after steroid therapy to myositis and resistant to steroid pulse therapy, on the contrary all those who had a good prognosis showed interstitial pneumonia before or when myositis occurred. In addition, change of viral titers for Coxsakie virus were detected in some patients. These facts may provide the possibility that the interstitial pneumonia in poor prognosis patients is not the complication of DM/PM itself but a viral infection. There were no differences in specific autoantibody titers such as Jo-1 between good and poor prognosis patients. As for the chest X-ray film, in most cases of both groups interstitial pneumonia began from lower lungs and seemed difficult to predict the prognosis from the radiological findings.

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Out of fifty-seven patient with systemic lupus erythematosus (SLE), we found eight patients (14%) with both antiphospholipid antibodies (aPL) and vascular occulusive diseases (VOD), and describe here the clinical features of eight SLE patients which were retrospectively analyzed.
Cerebral infarction and deep vein thrombosis were the frequent VOD observed. The other episodes of VOD include amaurosis fugax, mesenteric artery occulusion, transient ischemic attacks and pulmonary infarction. More interestingly, in multiple VOD were observed in 75% of the patients and the occurence of VOD was further associated with thrombocytopenia in all of the patients.
In addition, 4 (66.7%) of the six female patients with histories of pregnancy had recurrent spontaneous abortions which were also associated with thrombocytopenia.
Six (75%) of the eight patients were positive for more than 2 of the aPL including VDRL, lupus anticoagulants and anticardiolipin antibodies.
Treatments with prednisolone (PSL) and/or antiplatelet agents were considered to be effective to protect the recurrence of VOD. Spontaneous abortions, however, could not be protected by the treatment with PSL, 20-30mg/day, in our patients.

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We examined the association of antiphospholipid antibodies (aPL), including VDRL, anticardiolipin antibodies (aCL) and lupus anticoagulants (LA), with clinical features of 57 patients with systemic lupus erythematosus (SLE). In addition, the effects of prednisolone (PSL) treatment on levels of the antibodies were retrospectively determined in this study.
Out of the 57 patients with SLE, VDRL, aCL and LA were detected in 45.6%, 26.3% and 54.4%, respectively. A significant correlation was found between the result of VDRL and that of LA.
Activated partial thromboplastin time (a-PTT) significantly prolonged in the patients with positive result of either VDRL or LA. Thrombocytopenia or the other clinical feature of aPL syndrome such as recurrent abortions and vascular occulusive diseases was significantly frequent in the patients with LA. Furthermore, we found that the numbers of aPL detected were correlated with those of the clinical features of aPL syndrome.
Titers of the three aPL (VDRL, aCL and a-PTT) were relatively stable in patients with antiplatelet agent or non-steroidal anti-inflammatory drugs. However, PSL treatment, higher than 30mg/day, consistently lowered the titers of each aPL.
These results indicate that aPL should measured by different methods for the diagnosis and management of aPL syndrome, and that PSL, higher than 30mg/day, is effective when aPL titers must be lowered.

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The immunological mechanisms which prevent maternal rejection of the fetus have not yet been fully elucidated. Thus, it is very important to clarify the immunological environment of the decidual tissue existing at the interface between the fetus and the mother. In this study, the characteristics of mononuclear cells (MNC) infiltrating the decidual tissue (D-MNC) were investigated by using monoclonal antibodies specific for various surface antigens of MNC. In addition, the functions of D-MNC were also studied.
In comparison to peripheral blood MNC (P-MNC), in D-MNC, CD8⁺ cells were more numerous and CD4⁺/CD8⁺ ratio was decreased. Furthermore, two-color staining showed that 81% of CD8⁺ cells were CD11b^- cells. CD14⁺ cells were also increased. Immunohistological studies showed that CD3⁺ cells were present around the epithelial glands at the decidua spongiata, but not in the decidua compact. CD8⁺ cells were observed in greater number than CD4⁺ cells, and these CD8⁺ cells were CD11b^- cells. However, IL-2 receptor positive cells were not seen.
The functions of D-MNC were compared with that of P-MNC. Functions evaluated in this studies were PHA response, IL-2 response, suppressive activity and natural killer activity. The PHA reactivity of D-MNC was lower that of P-MNC, although the IL-2 response and suppressor activity of D-MNC was higher. Therefore, the activity of T-cells in the decidua was high, but suppressor cells and suppressor factors derived from decidual cells suppressed the function of D-MNC. The NK activity of D-MNC was lower than that of P-MNC.
In conclusion, although cytotoxic T-cells were more numerous in the decidua compacta than in peripheral blood, the action of suppressor cells and suppressor factors was consideredto prevent these cells from rejecting the fetus.

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Changes of lymphocyte subset phenotypes and lymphocyte response of phytohaemag-gulutinin (PHA) were studied in 12 patients with breast adenocarcinoma and compared between patients with positive lymph node (n=4) and negative lymph node metastasis (n=8).
In patients with positive lymph node metastasis, the response against PHA was significantly elevated on the 7th postoperative day when compared to patients with negative lymph node metastasis (p<0.05). CD4⁺CD29⁺T cells were significantly increased on the 7th day postoperatively in patients with negative lymph node metastasis (p<0.05), while, in patients with positive lymph node metastasis, number of these cells remained at low levels (p<0.05). CD8⁺⁺ Tcells with stronger expression of the CD8 antigen were at low levels on the 7th postoperative day in patients with positive lymph node metastasis when compared to patients with negative lymph node metastasis (p<0.05). CD8⁺⁺ CD11⁺ Tcells inpatients with positive lymph node metastasis, however, were significantly increased postoperatively compared to the preoperative level (p<0.05), and it was suggested that cytotoxic T cells were remarkably reduced.

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In order to understand the role of cytoplasmic oxygen radicals in allergic reaction, we tested purified eosinophils and neutrophils obtained from patients with or without allergic attacks as well as from non-allergic controls. The test methods included luminol dependent chemiluminescence, superoxide anion production and hydrogen peroxide production. Stimulation with phorbol 12-myristate 13-acetate induced higher production of the oxygen radicals in eosinophils than in neutrophils of normal controls in all three parameters. Eosinophils of allergic subjects in stable conditions behaved indistinguishable from that of normal controls. However, eosinophils obtained from patients during attacks of bronchial asthma were more responsive than those from patients between attacks. Neutrophils of patients during or between attacks showed the same profiles. The results suggested that the oxygen radicals released from eosinophils may have a significant role to injure the tissue during the bronchial asthma attack.

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Bucillamine [Bc; N-(2-mercapto-2-methylpropionyl)-L-cysteine] is one of the anti-rheumatic drugs which was developed in Japan. Interstitial pneumonitis induced by Bc has been reported in one patient with rheumatoid arthritis (RA). In this report, we describe another patient with RA who developed Bc-induced interstitial pneumonitis.
A sixty-two year-old female had developed RA since 1983. She had histories of adverse effects of anti-rheumatic drugs such as gold sodium thiomalate (genaralized skin rash) and D-penicillamine (fever). Although she had been well controlled by salicylazosulphapyridine since June, 1986, her disease relapsed on Jan., 1990. The treatment with Bc (200→300mg/day) was started on April, 1990. Her disease activity improved 3 months after the begining of Bc, but she developed dyspnea on July, 1990. She had hypoxemia (PO₂60Torr). Chest X-ray studies, pulmonary function tests and ⁶⁷Ga-scingaraphy of the lung were compatible with active, diffuse interstitial pneumonitis. Lymphocyte stimulation test using Bc was negative, and CD4/CD8 ratio in bronchoalveolar lavage fluid lymphocytes was normal. Interstitial inflammation with mononuclear cell infiltrations was observed in the specimen biopsied from her lung. Her symptoms persisted after the termination of Bc, but prednisolone (50mg/day) rapidly improved her symptoms and laboratory test results.

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Progressive systemic sclerosis (PSS) developing after fifteen years exposure to asbestos and silicon is reported.
The patient was a 53 year-old male, was in building contractor.
Since 38 years-old, he was in contact with asbestos and silicon.
Raynaud's phenomenon, sclerodactyly and arthritis were detected at 51 years-old.
At 53 years-old, shortenen of breath upon effort was detected.
He was admitted to Juntendo University Hospital because these symptoms did not improve.
At admission, Velcro role was audible bilaterally at the lower chest and lung fibrosis was recognized by TBLB.
By skin biopsy of the anterior chest skin and finger, the diagnosis of PSS was made.
The relationship between PSS and exposure to asbestos and silicon was of interest and discussion was made.

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A 38 year-old woman was admitted to our hospital complaining of myalgia and muscle weakness in august 1988. Since 1984, she was taking quinidine and procainamide for the treatment of severe arrythmia. On admission, laboratory data revealed pancytopenia, increased erythrocyte sedimentation rate, hypergammaglobulinemia, increase of CPK and aldolase. APTT was prolonged and mixing test was positive, indicating the presence of lupus anticoagulant. LE-test and LE cell were positive and ANF was increased to 5, 120×. The staining pattern of ANF was mainly homogeneous type. Western blot analysis using purified histone from calf thymus revealed the presence of IgG autoantibodies reacting with H1, H2B and H2A. Other autoantibodies such as PAIgG, anti-ssDNA and anti-cardiolipin antibodies were positive. Drug induced SLE like syndrome was suspected and administration of quinidine and procainamide was stopped soon after the admission. However, SLE like symptoms did not change for the first one month. Then, administration of prednisolone (40mg/day) was initiated. Although prednisolone administration was very effective for subjective symptoms at the beginning, recurrence of SLE like symptoms occured during the tapering of prednisolone. Long term administration of prednisolone for more than 6 months was needed for the control of the symptoms. The association of quinidine and procainamide with SLE-like syndrome is discussed.

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A case of Sjögren's syndrome complicated mainly with interstitial pneumonia in which high dose methylprednisolone therapy was effective was reported. A 60-year-old woman has been complaining of oral sicca syndrome, exertional dyspnea and general fatigue since early spring in 1990. Fine crackeles were audible and acceleration of ESR, hypoxia and restrictive ventilatory disorder were observed and a chest X-ray film showed “ground glass” shadows at bilateral lower lung fields. The diagnosis of Sjögren's syndrome with interstitial pneumonia was comfirmed by the presence of keratoconjunctivitis and also by lip and lung biopsy. Interstitial pneumonia was responsive to high dose methylprednisolone therapy in the present rare case which was characterized mainly by severe respiratory symptoms. And high dose methylprednisolone therapy was thought to be effective for these patients.

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We report a 27-year-old female of severe hypersensitivity vasculitis, who was successfully treated with immunoadsorbent therapy. She complained of purpura, polyarthralgia and cough in September, 1988. She was admitted to our hospital because of the deterioration of these symptoms and the following laboratory data became available.
1. There was palpable purpura on the bilateral legs and arms. Skin biopsy showed leukocytoclastic vasculitis.
2. Hb was 7.2g/dl, ESR 100mm/hr, PaO₂ 66mmHg. Abnormal serum values were IgG 3, 865mg/dl, immune complexes 61μg/dl and hypocomplementemia.
3. Renal biopsy showed granular deposition of Clq and IgG within the vessel walls of capillaries.
4. Respiratory function test revealed the decrease of % FEV1.0 and DLco. Her chest X-ray depicted reticular shadow in the bilateral leg base.
She was diagnosed as having severe hypersensitivity vasculitis with multiple organ involvement. She was treated with prednisolone 60mg/day but her clinical course was complicated with pulmonary hemorrhage. Immunoadsorption therapy with PH-350 column (Asahi Medical) was carried out to lower the serum level of immune complexes. After the immunoadsorption, treatment with prednisolone was continued and her condition got better.
Immunoadsorbent therapy using column with immobilized phenylalanine as ligand was effective for severe hypersensitivity vasculitis with high level of serum immune complexes and the involvement of multiple organs.

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A case of Sweet syndrome (acute febrile neutrophilic dermatosis) is reported in a patient with effective treatment by colchicine.
In 1964, R. D. Sweet reported eight patients with distinctive and fairly severe illness which refered to acute neutrophilic dermatosis. It is characterized clinically by a various combination of systemic and cutaneous manifestations. The principal features included fever, increased erythrocyte sedimentation rate, polymorphonuclear leukocytosis and raised, tender, erythematous plaques or nodules on the limbs, face and neak. In addition, histological examination shows a dense infiltrate of mature polymorphonuclear leukocytes in the edematous dermis.
A dramatic response to systemic corticosteroids is one of the characteristic features of acute febrile neutrophilic dermatosis. In most of the cases, corticosteroids successfully controlled the disease, but a rapid withdrawal of the medication may frequently cause the recurrence.
In this article, we report one patient of Sweet syndrome who showed dramatic and rapid response to systemic administration of low dose colchicine.

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A 36 year-old man was admitted because of diplopia and decreased memory. He had been homosexual since 22 years old. Before this admission, he had already presented symptoms of immunodeficiency, such as viral and fungal pneumonia, oral candidiasis and pneumocystis carinii pneumonia. A positive anti-human immunodeficiency virus (HIV) antibody was confirmed by immunofluorescence assay.
On admission, he had typical symptoms of acquired immunodeficiency syndrome (AIDS) related dementia complex, including decreased memory, the difficulty to concentrate, apathy and psychomotor retardation. Neurological findings revealed the right abducens paralysis, defect of temporal visual field and bilateral plantar dysesthesia. Ophthalmoscopic examination showed degenerative change in the right nasal retina.
Immunological findings demonstrated severe immunodeficiency: CD4 1.6% (3/mm³), CD4/CD8=0.03, decreased mitogenic response (Phytohemagglutinin 3, 326cpm, Concanavalin-A 821cpm, Control 275cpm). Progressive brain atrophy was disclosed with computed tomography. Decreased perfusion in whole brain especially in parietal and occipital lobes was displayed on brain perfusion scintigram. Electroencephalography showed the sleeping wave.
Various therapeutic agents including azidothymidine (AZT), γ-globulin, glycyrrhizin, interferon α and neurotropin were not effective. He died of respiratory arrest due to cytomegalovirus pneumonia or damage of central nervous system.
During the course of illness, he also presented eosinophilia, polyclonal gammopathy, thrombocytopenia and hemolytic anemia.

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This paper discusses a case of immunoblastic lymphadenopathy (IBL)-like drug allergy with elevated serum intrerleukin 6 (IL-6) activity. The patient is a 43-year-man. He developed fever after having received an antipyretic agent, followed by systemic lymph node swelling, skin rash, and hepatosplenomegaly.
Furthermore, the patient also presented with clinical symptoms reminiscent of IBL, such as marked hemolytic anemia, polyclonal hypergammaglobulinemia, positive reactions for various kinds of auto antibodies, and cellular immunosuppression. However, the pathological picture of the resected lymph node was incompatible with IBL but compatible with reactive lymphadenitis, and the patient gave a strong positive reaction to drug stimulation of lymphocytes. Based on these findings, a diagnosis of IBL-like drug allergy was made. In an attempt to elucidate the etiopathologies of this disease IL-6 activity in the serum and lymphocyte culture supernatant was determined. As a result, IL-6 activity was detected in serum which is normally free of it and was also elevated in lymphocyte culture supernatant. This finding is of interest from an etiopathological standpoint.

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A 50-year-old man was admitted to the hospital because of acute onset polyarthritis, which involved his bilateral ankles, wrists, elbows and left knee. He also had back pain and a limited range of flexure in his lumbar column. He was HLA B27 positive and rheumatoid factor negative with a highly elevated erythrocyte sedimentation rate (ESR) of 158mm/h. No radiological changes were seen in the sacroiliac (SI) and other involved joints, except small osteophytes in lumbar spine. Bone scintigraphy revealed increased uptake in bilateral ankles, wrists, the first right MP, and the second right PIP joints. Ankylosing spondylitis was suspected but not diagnostic because of his intact SI joints. During 5 years follow up, his polyarthritis and spinal symptoms exacerbated several times with constitutional signs. However, no abnormal radiological findings were obtained in lumbar spine, SI joint, and peripheral joints.
Our patients was characterized by late onset, positive HLA B27, minimal axial symptom, moderate peripheral non-erosive arthritis with elevated ESR, and no radiological changes in spine and SI joint. These findings were considered to be compatible with “late onset peripheral spondyloarthropathy” described recently by Dubost and Sauvezie.

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We report a case of polyarteritis nodosa comlicated with massive perirenal hematoma and gangrene of distal extremities. A 69-year-old male was admitted to Tsukuba University Hospital, complaining of fever and pain of fingers and toes. Physical examination revealed a right abdominal mass and gangrene of the finer tips and toes. Leukocytosis with eosinophilia and an elevated erythrocyte sedimentation rate were observed. Urinalysis revealed proteinuria and hematuria. Tests for antinuclear antibodies and blood cultures were all negative. Selective angiography of the renal arteries revealed intraparenchymal multiple microaneuryms, small arterial obstructions and wedge-shaped infarctions. An enhanced abdominal CT showed right perirenal hematoma. The clinical diagnosis of polyarteritis nodosa was made on the basis of the combination of clinical pictures and angiographic findings. Steroid therapy was initiated and the patient recovered rapidly.

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