We experienced a case of Takayasu's arteritis (TA) with ulceritive colitis (UC) having the onset of carotodynia, not accompanied by ischemic symptoms. MRI findings of the neck demonstrated a thickening of the bilateral carotid artery walls. The patient was treated by prednisolone with a marked improvement in both clinical symptoms and MRI findings. The patient had a unique HLA haplotype reported to correlate with both TA and UC. Carotodynia is an early but pathognomonic symptom of TA and MRI is helpful for the early diagnosis of TA.

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A case of vasculitis syndrome associated with epididymitis is reported. A 56-year-old male presented with the sudden left testicular pain and fever. He came to the department of urology in our hospital, and was treated with antibiotics and anti-tuberculous drugs. However, the testicular pain was not relieved as well as the fever. In January 5, 1993, he was admitted. He had lost 6kg. Physical examination revealed pyrexia and blood pressure of 150/91mmHg. Laboratory finding revealed leukocytosis, thrombocytosis, and erythrocyte sedimentation rate of 100mm/hr. On serological examination, the C-reactive protein was positive. We suspected this case as a polyarteritis nodosa, then he was given prednisolone. On January 20, 1993, left orchidectomy and spermatic cord biopsy was performed and non-necrotizing vasculitis was found. On August 10, cyclophoaphamide was added and his symptom disapeared. Although polyarteritis nodosa is well known as the vasculitis affecting the epididymis, it was rare that initial manifestation was testicular pain. In this case necrotizing vasculitis was not proven, but we diagnosed this case as a vasculitis in which the initial symptom was the epididymitis.

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We report a case of IgM-λ type monoclonal gammopathy of undetermined significance showing non-specific anti-streptolysin O activity of extremely high level. An 83-year-old man developed high grade fever, cough and sputum. He was admitted to our hospital under a diagnosis of acute pneumonia by chest X-ray. He showed anti-streptolysin O (ASO) activity in extremely high level measured by a latex immunoaggregation method (LA-method). Although antibiotics cured the acute pneumonia, the ASO activity had remained in high level. Serum protein electrophoresis disclosed existence of M-protein and the M-protein was found to be IgM, λ class by immunoelectrophoresis. The ASO activity measured by the LA method was ascribed to the M-protein because the level of M-component shown by the electrophoresis was decreased by absorption of the serum with streptolysin O-coated latex beads that were used in the LA-method. However, ASO activity measured by Rantzs-Randall method was in normal level. The ASO activity measured by the LA-method was absorbed with bovine serum albumin coated latex beads without streptolysin O. Therefore, it was concluded that the M-protein was not reacted with streptplysin O itself but was reacted with bovine serum albumin coated latex beads. The ASO activity of extremely high level in our case was non-specific reaction caused by the M-protein.

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Systemic lupus erythematosus (SLE) is an autoimmune collagen vascular disease which produces widespread damage to multiple organs. Few studies on laryngeal involvement in SLE have been reported. We report here a case of SLE complicated by lupus laryngitis. A 27-year-old woman was diagnosed as having SLE in October 1996 based on findings of polyarthritis, lymphocytopenia, positive anti-nuclear antibody and anti-Sm antibodies. Polyarthralgia disappeared transiently, and no other clinical symptoms were found. Therefore, she was followed with no medication.
She presented hoarseness with high fever and facial erythema in September 1998. Laboratory findings on admission revealed pancytopenia and hypocomplementemia. Anti-nuclear antibody, anti-Sm antibody and anti-RNP antibody were all positive with high titers. Chest X ray examination showed pleural effusion.
Laryngoscopy showed a bamboo-joint-like lesion at the middle of the bilateral vocal cords. She was treated with 60mg of prednisolone (i.v.) which resulted in improvement of hoarseness, pancytopenia, hypocomplementemia and pleuritis. Based on these findings, we diagnosed her hoarseness as the manifestation of laryngitis associated with SLE (lupus laryngitis).

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A 46-year-old female was admitted to our hospital due to general fatigue, systemic edema and dyspnea with history of systemic sclerosis (SSc). The patient was diagnosed as mixed connective tissue disease (MCTD) based on Raynaud phenomenon, a high anti-RNP antibody level and clinical symptoms and laboratory findings suggesting SSc, dermatomyositis (DM) and systemic lupus erythematosus (SLE). After the admission, both alveolar hemorrhage and a rapidly progressive glomerulonephritis (RPGN) also developed and laboratory findings showed a positive remark of myeloperoxydase-antineutrophil cytoplasmic antibody (MPO-ANCA) and anti-glomerular basement membrane (GBM) antibody. She was therefore re-diagnosed as microscopic polyarteritis nodosa (microscopic PAN) combined with MCTD and treatment with high dose prednisolone and steroid pulse therapy dramatically improved general conditions and lung symptoms, but maintenance dialysis was persistent because of irreversible renal failure. However, 3 months after the admission, she died of acute exacerbation of interstitial pneumonitis that was unresponsive to steroid pulse therapy. Autopsy revealed interstitial pneumonitis with alveolar hemorrhage and crescentic glomerulonephritis (CrGN), in which immunofluorescent microscopy showed no deposition in agreement with pauciimmune type. The histological findings supported the diagnosis; primary microscopic PAN combined with MCTD, which is a quite rare case, to our knowledge. Furthermore, co-existence of MPO-ANCA and anti-GBM antibody, clinical and histological findings of the case also lead us to reconsider the relevance of these antibodies to pathogenesis and/or categories of microscopic PAN and Goodpasture's syndrome.

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We described an 11 year-old boy with systemic lupus erythematosus (SLE) and various coagulopathy. He had purpura on the legs, pancytopenia, positive anti-DNA antibodies and hypocomplementia. Hematological examination also showed that platelet counts were 80×10³/μl, lupus anticoagulant and anticardiolipin antibodies were positive. The aPTT was remarkably prolonged. Those laboratory findings fulfilled the criteria of antiphospholipid syndrome. Following treatment with predonisolone and heparin, thrombocytopenia improved. When heparin discontinued and renal biopsy was performed, severe thrombocytopenia recureded. FDP and FDP-DD became high, but the aPTT was not prolonged. Thrombocytopenia didn't improved by the therapy with heparin, high dose of methylpredonisolone, FOY and γ-globulin. However by the therapy with both warfarin and cyclophosphamide, remarkable improvement of coagulopathy was absorbed. Probably anticardiolipin antibodies and disseminated intravascular coagulation (DIC) participate in the various coagulopathy in this case.

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We present here a case of MPO-ANCA positive rapidly progressive glomerulonephritis (RPGN) after 34 months of D-penicillamine (D-PC) therapy for rheumatoid arthritis (RA).
A 27-year-old Japanese woman was diagnosed as having RA in June 1994 at our outpatient clinic. Oral D-PC administration was initiated at a dose of 100mg per day in January 1995. In August 1997, proteinuria, hematuria, renal insufficiency, and anemia developed. D-PC was withdrawn promptly, and prednisolone 5mg per day was started. The patient was admitted to our hospital in September. On admission, anti-neutrophil cytoplasmic antibody against myeloperoxidase (MPO-ANCA) was strongly positive in the serum. Renal biopsy showed glomerulonephritis with cellular crescent formation in 60% of the glomeruli observed. Immunofluorescence examinations revealed deposits of granular IgG, IgA, C1q, and C3 in the mesangium. The patient was treated with steroid pulse therapy along with administration of anti-coagulation and anti-platelet agents under the diagnosis of MPO-ANCA positive D-PC-induced RPGN. The renal function was gradually recovered and MPO-ANCA disappeared.
Since RPGN is potentially a fatal disease, frequent monitoring of renal function and discontinuation of D-PC are required. In case MPO-ANCA becomes positive, prompt and correct diagnosis of the renal disorder could lead to a good prognosis as in this case.
The present case may provide some important immunological insights into medical procedures to treat D-PC-induced RPGN and MPO-ANCA related glomerulonephritis.

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