Ryumachi. [Rheumatism]最新文献

We investigated to clarify the clinical findings, course and therapeutic effect in the patients with MPO (myeloperoxidase)-ANCA (anti-neutrophil cytoplasmic antibody) associated vasculitis syndrome. We analyzed clinical findings and data of 19 cases of MPO-ANCA associated vasculitis. These patients were diagnosed with clinical symptoms (fever, arthralgia, body weight loss, etc.), laboratory data (high titer of CRP, leukocytosis, thrombocytosis, and high titer of MPO-ANCA) and pathologic findings of necrotizing vasculitis. They were 14 male and 5 female aged 18 to 84 years (mean 65 years) and were treated with prednisolone and immunosuppressive agents, and additional therapy included pulse therapy and plasma exchange. Seven cases were dead within 3 months. Post-mortum examination showed that these cases died of pneumonitis, cerebral events and gastric bleeding. There was no mortal case induced by over-immunosuppression. In survival cases, the MPO-ANCA levels decreased rapidly after these therapies and these antibodies were maintained low levels (360 to 25 EU/l). Comparison of fatal cases and survival cases, there were difference in the initial dose of prednisolone (27 mg/day vs. 56 mg/day), the ratio of double filtration plasmapheresis (14% vs. 42%), and the ratio of immunosuppressive therapy (14% vs. 83%). The measurement of MPO-ANCA is useful makers of the diagnosis and effectiveness of the therapy in patients with MPO-ANCA associated vasculitis. We recommend the aggressive therapy, including prednisolone, immunosuppressive agents and plasma exchange for MPO-ANCA associated vasculitis. We believe that the aggressive therapy improve the survival rate of the patients with MPO-ANCA associated vasculitis.

We studied two autopsy cases, each with a low titre of myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) associated with systemic vasculitis. It was difficult to distinguish these cases from classic polyarteritis nodosa. The patients had suffered from continuous fever, malaise, and weight loss: however, their renal insufficiency was clinically mild over the course of their disease. The patients were diagnosed initially as having MPO-ANCA-associated vasculitis and were treated with prednisolone. Their clinical status improved, but unfortunately, they died of an infectious disease. Autopsies revealed systemic vasculitis in small arteries with no signs of necrotizing and crescentic glomerulonephritis. Our pathologist subsequently diagnosed both cases as classic polyarteritis nodosa. Systemic vasculitis associated with MPO-ANCA is usually considered to be a microscopic polyarteritis. However, classic polyarteritis nodosa should always be considered as a possibility for those patients with mild renal insufficiency and a low titre of MPO-ANCA.

A 55-year-old male was admitted to our hospital because of arthralgia, rash, and painful tumor in the legs. On admission, skin lesions involving dark erythematous macules and a tender mass of 3 by 5 cm were present. Laboratory findings included hemoglobin of 11.2 g/dl, white blood cell count of 6200/microliter, erythrocyte sedimentation rate (ESR) of 88 mm/hour, and normal results of renal function tests. Hepatitis B surface antigen, anti-nuclear antibody (ANA), and perinuclear pattern antineutrophil cytoplasmic autoantibody (ANCA) were negative. A T 1-weighted magnetic resonance image (MRI) showed iso-intensity areas that increased in intensity on T 2-weighted images of the M. gastrocnemius. The angiographic appearance of tumor staining with some degree of luminal irregularity in the posterior tibial artery suggested a diagnosis of soft tissue neoplasm. Biopsy specimens of the right leg lesion were consistent with necrotizing arteritis of both small and medium-sized vessels. Treatment with prednisolone and cyclophosphamide resulted in the disappearance of the skin lesions and the improvement of laboratory data. At first, classical polyarteritis nodosa was considered histologically. However, lesions were limited to skin, muscles, and joints, and there has been no evidence of systemic disease for 1.5 years; consequently, cutaneous form of polyarteritis nodosa was diagnosed. Reports of soft tissue tumors on the legs of patients with polyarteritis nodosa are quite rare. The interesting radiographic findings of cutaneous polyarteritis nodosa were reported.

Objectives: This study was performed to investigate the efficacy and safety of cyclosporin A (CsA) for the treatment of interstitial pneumonia (IP) associated with collagen diseases in Japan.

Methods: Questionnaires were sent to 36 hospitals specializing in collagen diseases.

Results: Fifty-eight patients (7 polymyositis (PM), 19 dermatomyositis (DM), 7 systemic sclerosis (SSc), 7 rheumatoid arthritis (RA), 2 mixed connective tissue disease (MCTD), 1 systemic lupus erythematosus (SLE) and 1 Sjögren's syndrome (SS), 1 RA + SSc, 2 PM + SSc, 1 DM + SLE, and 10 idiopathic interstitial pneumonia (IIP) with IP were treated with CsA at 14 hospitals. IP was classified into the acute or chronic type. In the PM/DM group (7 PM, 19 DM, 2 PM + SSC, 1 DM + SLE), 65.5% were the acute type. In the other collagen disease group (7 SSc, 7 RA, 2 MCTD, 1 SLE, 1 SS, and 1 RA + SSc) and IIP group, 36.8% and 50% were the acute type, respectively. Mean dosages of CsA were 3.7 +/- 1.3 mg/kg/day for the PM/DM group, 3.0 +/- 1.0 for the other collagen disease group, and 3.8 +/- 4.8 for the IIP group. Oral corticosteroids were administered in combination with CsA in 100, 73.7, and 70% of the patients with PM/DM, other collagen disease, and IIP groups, respectively. CsA was effective for 72.2, 33.3, and 25% of the acute IP cases in the PM/DM, other collagen disease, and IIP groups, respectively. CsA was effective for 50.0, 50.0, and 60.0% of chronic IP cases in the PM/DM, other collagen disease, and IIP groups, respectively. Twenty-three adverse effects were observed, but most of them ameliorated upon withdrawal or reduction of the CsA dose.

Conclusion: CsA is effective for the treatment of acute type IP associated with collagen diseases, especially PM/DM. To perform a prospective multi-center trial, standards for the recruitment of patients, efficacy assessments, and trial course and treatment should be determined carefully.

A 52-year-old woman, diagnosed as having Sjögren's syndrome by parotid sialography and lip biopsy after a two years history of recurrent purpuric rashes on her lower extremities, was admitted to our hospital because of visual disturbance in March 1998. On presentation at the department of ophthalmology, her right visual acuity was light perception, and laboratory findings showed elevated levels of antinuclear antibody and anti-Ro/SS-A and anti-La/SS-B antibodies. Cerebrospinal fluid analysis showed mild pleocytosis and elevated levels of total protein and Q-albumin. The IgG-index was within normal level and no oligoclonal band was found. Magnetic resonance imaging showed increased signal intensity at the right optic nerve. After treatment with m-PSL pulse therapy, her visual acuity recovered to 0.08. When prednisolone was gradually tapered to the dose of 30 mg per day, she was transferred to our department because of high grade fever and pancytopenia. She also suffered from palpable purpura in her extremities extending the trunk, whose pathological diagnosis was leukocytoclastic vasculitis. The immunohistochemical examination showed depositions of IgG and C1q. After two additional cycles of mPSL pulse therapies, clinical improvement was achieved. The titers of von Willebrand factor and thrombomodulin correlated with her clinical improvement. Patients with Sjögren's syndrome can develop extra-grandular complications, including neurologic and cutaneus diseases, it is important to understand the role of SS-A-B antibodies in the immunopathogenesis of Sjögren's syndrome.

A 43-year-old man was admitted to a hospital because of acute dyspnea and nocturnal orthopnea. Echocardiogram and chest CT showed the dilation of thoracic aorta from the root to ascending portion. On the third hospital day, he died suddenly. At autopsy, the cause of death was indicated to be a tear of an aortic valve due to a rupture of the aneurysm of Valsalva's sinus, followed by acute aortic regurgitation and acute cardiac insufficiency. Histopathological findings of thoracic aorta revealed mesoaortitis, characterized by patchy destruction of the media with a moth-eaten appearance of the medial elastic laminae and a microgranuloma formation, a perivascular mononuclear cell infiltration of the vasa vasorum, and a fibrous thickening of the intima and adventitia. However, there were no abnormalities in main branches of aorta and abdominal aorta, and no systemic vasculitis. This case is a rare one in the clinical course, and may be important to be differentiated from other cases with aortitis, especially Takayasu arteritis and syphilitic aortitis.

Objectives: We investigated the effect of the combination therapy of prednisolone (PSL) and immunosuppressants after methylprednisolone pulse therapy.

Methods: A protocol of PSL (15-20 mg/day) and mizoribine (150-200 mg/day) after methylprednisolone (mPSL) pulses was used for 2 years to treat 7 patients (PSL + MZB group). Cyclophosphamide (CYC) pulse therapy was added to the combined therapy in 4 patients with severe lupus nephritis. The total dose of predinisolone, and side effects were compared with those in 6 patients who were treated with PSL (30 mg/kg) alone after mPSL pulse therapy (PSL group).

Results: No relapses occurred in the PSL + MZB group, although all of 6 patients relapsed in the PSL Group. The total doses of PSL in the PSL + MZB group was about 70% of the PSL Group. There were two patients with Herpes-Zoster infection and one patient with liver dysfunction as side effects, with no differences in the frequency of side effects between the was groups.

Conclusions: Combination maintenance therapy with prednisolone and immunosuppressants after methylprednisolone pulse therapy was effective in preventing relapse.