Kazuhide Yamane

Clinical Features of Patients with Mild Systemic Lupus Erythematosus

Clinical features of 16 patients with mild systemic lupus erythematosus (SLE) were compared with those of 21 control patients with moderate or severe disease. Age at the time of diagnosis of SLE was higher in mild disease group. The incidence of the coexistence of Sjögrens syndrome (SS) at the time of diagnosis of SLE was higher in patients who later developed mild disease. Anti-Sm antibody and decreased levels of C3, C4, and CH50 occurred less frequently in patients with mild disease. SLE patients with the coexistence of SS at the time of diagnosis of SLE may represent a subset with a benign prognosis.

Necrotizing fasciitis caused by Streptococcus pneumoniae in mixed connective tissue disease

Abstract A 42-year-old man was hospitalized because of chills, fever, and severe polyarthralgia. He had a 5-year history of mixed connective tissue disease (MCTD) with polyarthritis which had been treated with a nonsteroidal anti-inflammatory drug alone. On the second day of hospitalization, necrotizing fasciitis of the right leg developed. Streptococcus pneumoniae was later detected in a blood culture. Pneumococcal infections of the soft tissues are uncommon, and have been reported in immunosuppressed patients and patients with systemic lupus erythematosus. This is the first report of a case of necrotizing fasciitis caused by S. pneumoniae in a patient with MCTD.

Monocyte-mediated suppression of T lymphocyte blastogenesis and its reversal by deoxyguanosine. Defects in patients with systemic lupus erythematosus

We investigated monocyte-mediated suppression of T lymphocyte blastogenesis in normals and patients with systemic lupus erythematosus (SLE). When monocytes from normals were cocultured with autologous T lymphocytes with a ratio of 1:1 and stimulated with phytohemagglutinin (PHA), 3H-thymidine incorporation by T lymphocytes was suppressed. This monocyte-mediated suppression was reversed by purine nucleoside phosphorylase substrate, deoxyguanosine. In SLE patients, both monocyte-mediated suppression and its reversal by deoxyguanosine were defective. The defective function was observed both in patients with active and inactive diseases. The defective function was studied sequentially before and after change in the clinical status of patients. The defects remained unaffected regardless of the disease activity. The defects in monocyte-mediated suppression and its reversal by deoxyguanosine in SLE patients as demonstrated in our study suggest the presence of intrinsic monocyte dysfunction in SLE.

Gelatinous transformation of bone marrow with pancytopenia in an emaciated patient with systemic lupus erythematosus

We report on gelatinous transformation of the bone marrow in a 42-year-old male patient with systemic lupus erythematosus (SLE). Gelatinous transformation of the bone marrow in SLE is rare. This condition is composed of hypocellularity with deposition of hyaluronic acid in the bone marrow and is known to be associated with malnutrition due to cachexia and anorexia nervosa. In this patient we consider that malnutrition caused gelatinous transformation which worsened pancytopenia due to SLE. Pancytopenia improved after methylprednisolone pulse therapy and nutrition therapy.

A case of giant cell arteritis simulating aortitis syndrome : A case report.

73才の女性で,巨細胞動脈炎に甲状腺機能亢進症と糖尿病を合併した症例を経験した.大動脈弓部動脈群を侵す巨細胞動脈炎と大動脈炎症候群との鑑別には,発病年令,頭痛,側頭動脈の圧痛,肺・腎血管病変の有無, polymyalgia rheumaticaの合併の有無,抗大動脈抗体の存在およびその抗体価, HLAなどが参考になると考えられる.診断上,生検は必須であるが,病変が分節的に側頭動脈以外の小動脈にも及ぶことがある.甲状腺機能亢進症を合併していたが,両者の合併の背景には自己免疫学的な機序の関与が示唆された.

A CASE OF OSLER'S DISEASE WITH GASTRODUODENAL ULCERS

症例は65才,主婦.鼻出血,心窩部痛を主訴に入院した.父に鼻出血,娘に鼻出血および毛細血管拡張病変を認めた. 14才頃より鼻出血が時々あり, 57才頃より毛細血管拡張と口腔内出血が著しくなつた.入院時,皮膚,口腔・鼻腔粘膜の著しい毛細血管拡張,眼瞼結膜に強度の貧血を認めた.肺には異常血管雑音を聴取せず,肝は約4cm触知したが,脾腫は認めなかつた.検査所見では便潜血陽性,ヘモグロビン6.5g/dl,血清鉄, UIBCは鉄欠乏型であつた.肝機能障害を認め,超音波検査は肝硬変に一致した.凝固系検査は正常であつた.胃・十二指腸造影検査,内視鏡検査にて胃および十二指腸潰瘍を認め,潰瘍底部とその周辺部に毛細血管拡張病変を認めたこと,胃液検査にて胃酸が分泌過多であつたことから,本症における消化性潰瘍の発生機序について考察を加えた.