A clone of type 2 T cells (T6S cell) was established from burn-associated CD 8⁺ CD 11 b⁺ TCRγ/δ⁺ type 2 T cells, and their biological properties were examined. After stimulation by anti-CD 3 monoclonal antibody (mAb), T6S cells produced IL-4 and IL-10 into their culture fluids. However, IFN-γ and IL-2 were not demonstrated in the culture fluids of T6S cells stimulated with the mAb. The susceptibility of thermally injured mice to infection with herpes simplex virus type 1 (HSV-1) or Candida albicans (C. albicans) has been shown to be approximately 50 (C. albicans) to 100 (HSV-1) times greater than that of normal mice. As compared with the resistance of thermally injured mice to these pathogens, mice inoculated with 1×10⁶ cells/mouse of T6S cells (designated as T6 S-mice) showed the same susceptibility to the infections. When T6 S-mice treated with a mixture of monoclonal antibodies (mAbs) directed against IL-4 and IL-10 were infected with a lethal dose of HSV-1 or C. albicans, their resistance to these pathogens was restored to levels found in normal mice. From the experiments using T6 S-mice, it is suggest that type 2 T cells or their cytokine products (IL-4 and IL-10) may play an important role on the impaired resistance of thermally injured mice to certain opportunistic pathogens. T6S cell may be a useful tool to study opportunistic infections in hosts bearing type 2 T cells.

View full abstract

We describe a patient with systemic lupus erythematosus (SLE) with lupus psychosis, who showed prolonged consciousness disturbance due to hyperosmolality. A 51-year-old Japanese woman with SLE was admitted to our hospital for the evaluation and treatment of consciousness disturbance on March 5, 1994. She had not been given prednisolone since 1984, and had been depressive since January 1994. She was diagnosed as active SLE with lupus psychosis due to the presence of thrombocytopenia, proteinuria, positive anti-nuclear antibody (×10240) as well as the elevation of cerebrospinal fluid (CSF) IL-6 level. A treatment with methylprednisolone (mPL) 100mg/day was started along with 2 courses of steroid pulse therapy (mPL 1g/day for 3 consecutive days). She recovered partially from the central nervous system manifestations with a decrease in CSF IL-6 level 2 weeks after this treatment. However, her consciousness level was exacerbated again thereafter. Blood examination disclosed the elevation of plasma osmolality (319 mOsm/kg) with poor responses of plasma antidiuretic hormone (4.6pg/ml). She died from systemic aspergillosis on April 26, 1994. Pathological examination on autopsy showed no abnormality in hypothalamus and pituitary gland. It is suggested that this patient was complicated with lupus psychosis as well as hyporesponsiveness of osmoreceptor. Rheumatologists should be aware of this complication in patients with CNS lupus as a possible cause for intractable CNS manifestations.

View full abstract

A 72-year-old patient was admitted to our hospital because of painful tongue and loss of taste. A mediastinal mass on X-ray of the chest had been seen for 30 years, however no symptom was developed. Lichen planus was seen in his oral cavity. Hematological test showed decrease of all classes of immunoglobulin. CT and MRI of the chest showed a huge mediastinal mass. Needle biopsy was performed, giving rise to the diagnosis of thymoma with hypogammaglobulinemia (Good's syndrome). Intravenous immunoglobulin infusion, which is known to be effective to chronic diarrhea of this syndrome, improved his diarrhea. Lichen planus may be caused by T cell abnormality as suggested previously. Resection of the thymoma was not performed, since it does not improve hypogammaglobulinemia. This is a rare case of Good's syndrome with lichen planus.

View full abstract

Polyarteritis nodosa (PAN) is a multisystem inflammatory disease associated with necrosis of small and medium arteries. It can occur in a systemic form with manifestations in skin, joints, heart, nervous system, gastrointestinal tracts, lungs and kidneys. It is relatively rare in adults and even more in children. It maybe divided into a cutaneous form (C-PAN) and a more generalized form (G-PAN). Usually C-PAN is thought to run a benign course. But some cases were reported to run chronic courses and be diagnosed G-PAN. It has been reported that some adults with PAN are related to HBV infection. On the other hand most children with PAN had upper respiratory infections some with streptococcal infections. We described a 5-year-old boy who was suffering from high fever, joint pain, subcutaneous nodular and livedo reticularis. We diagnosed him C-PAN by examination and clinical course. Anti-streptolysin O (ASO) and anti-streptokinase (ASK) increased and we suspected that C-PAN followed after streptococcal infection. We reviewed the literatures of C-PAN and streptococcal infection in childhood. Streptococcal infection has been implicated by a positive throat swab or an increase in ASO. There were 25 cases (75.7%) which were thought to be related with prior streptcoccal infection in 33 cases. We thought that C-PAN may be associated with streptcoccal infection in childhood.

View full abstract

We describe the case of a 43-year-old woman who presented with primary Sjögren's syndrome (SS) which manifestated as unconsciousness due to M protein. A diagnosis of SS was made based on a ten-year history of dryness, a Shirmer test, and the histological findings of labial biopsy. A rouleaux formation was observed and serum protein electrophoresis revealed a monoclonal spike of 4.0gm/dl in the γ-region, which was characterized as IgA-λ. Biopsy of the minor salivary glands showed marked polyclonal lymphoproliferation with lymphoid follicles, including both T cells and B cells as revealed by immunohistological staining. Therefore, the patient had a lymphoproliferative lesion of the minor salivary glands, which is also known as pseudolymphoma. We conclude that fainting associated with marked M protein may be manifestation of SS. Such cases should be followed carefully since the subsequent neoplastic transformation of pseudolymphomas have been previously reported.

View full abstract

A 19-year-old woman with long-standing mixed connective tissue disease was admitted for dizziness. We examined cerebral blood flow quantitation using ^99mTc-hexamethylpropyleneamine oxime (HMPAO) and single photon emission computed tomography (SPECT) at rest and after cold pressor test. Mean cerebral blood flow reduced remarkably when she complained dizziness and showed peripheral Raynaud's phenomenon after cold exposure. We concluded cold-induced reversible brain ischemia was the reason of dizziness. Our finding suggests brain Raynaud's phenomenon. Further studies are necessary to clarify this phenomenon.

View full abstract

We described a 37-year-old man with Crohn's disease (CD) resembling systemic lupus erythematosus (SLE) at his disease onset. He was admitted to the municiple Akiru Hospital in October 1986 by fever, aphtous oral ulcerations, sore throat and polyarthralgia. Hematologic examination showed leukocytopenia, lymphocytopenia, positive tests for antinuclear antibody, anti-DNA antibody and LE cell phenomenon. He has had episodes of convulsion and conciousness loss of unknown etiology when he was 17 years old. The diagnosis of SLE was made, and oral medication of prednisolone was started. Several weeks later, most of symptoms and autoantibodies disappeared, although the oral aphtous ulcerations and leukocytopenia remained. In May 1987, he admitted to the other hospital because of bloody vomiting. Endoscopic examination showed the esophagial ulceration, and histology of biopsied-specimen was nonspecific esophagitis. The combination of prednisolone and oral cyclophosphamide or methotrexate was employed thereafter. However, the leukocytopenia, oral aphtous ulceration and esophagial ulceration continued in spite of these treatments. All the immunosuppressive treatment was stopped at March 1992. In October 1995, he admitted to our hospital because of body weight loss and continuous diarrhea with occasional bloody stool. Barium enema and endoscopic examination of the colon revealed the findings compatible with CD. The patient responded favorably to methylprednisolone pulse therapy followed by oral sulphasalazine.
This case indicated that cases with inflammatory bowel diseases like CD could show similar clinical signs and symptoms to SLE, and in some cases of CD might satisfied the classification of criteria for SLE.

View full abstract