Ryumachi. [Rheumatism]最新文献

A 15 year-old girl was admitted to the hospital because of fever, polyarthlargia, dry cough, dyspnea, butterfly rash and multiple oral aphthas. The diagnosis of systemic lupus erythematosus (SLE) was made based on renal disorders, pancytopenia, positive antinuclear antibody and positive for antibodies to double-stranded DNA. On admission, she developed progressive dyspnea with highly active SLE. The patient was complicated with both pulmonary hypertension (PH) and interstitial pneumonitis (IP), judging from increased pulmonary sound by an auscultation, interstitial shadows especially at bilateral lower lung and enlarged shadow of right atrium in a chest rentgenogram, ground glass pattern of bilateral middle to lower lung in a chest computed tomographic scan, increased pulmonary artery pressure, 53 mmHg, by an ultrasound cardiograph (UCG). Combination of methylprednisolone pulse therapy, cyclosporin A and plasma exchanges was effectively administered, which resulted in improvement of disease activity of SLE, IP and PH. However, two months later, although disease activity of SLE was completely reduced, recurrence of PH by UCG and multiple pulmonary embolism (PE) which was observed by a chest rentgenogram and a pulmonary blood flow scintigraphy was further complicated. Administration of cyclophosphamide pulse therapy and warfarin therapy improved both PE and PH. The patient had PH at the different clinical course of SLE; 1) PH maybe induced by severe IP at the active phase of SLE and 2) PH brought about from multiple PE at the inactive phase of SLE. Thus, the case is thought to be suggestive of elucidating the pathogenesis of PH of several systemic autoimmune diseases including SLE.

A 29-year-old woman experienced Raynaud's phenomenon, swelling of her fingers, eruptions on her face, and muscle weakness in 1990. She was diagnosed as having mixed connective tissue disease (MCTD) and was treated with prednisolone (PSL) for 3 years. Most of her complaints disappeared after the treatment. In March 1997, she experienced fever, erythema, and lymphadenopathy. Although she was treated with PSL (20 mg/day) again, muscle weakness, mental disturbance, and recto-urinary disturbance appeared. When she was re-admitted to our hospital, increased levels of muscle-derived enzymes and positivity of anti-RNP antibody were found. High signal areas in her cerebrum were shown by magnetic resonance imaging, and slow and spike pattern was shown by electroencephalography. Hypoperfusion of the cerebral blood flow was suggested on single photon emission computed tomography. The number of mononuclear cells, amount of protein and level of interleukin-6 were found to be elevated in her cerebrospinal fluid (CSF). Her neurological manifestations were diagnosed as being due to MCTD, and showed characteristics similar to those of systemic lupus erythematosus. She was treated with PSL (60 mg/day) followed by steroid pulse therapy. Because the response to this treatment was partial, oral administration of cyclophosphamide (CPM) (100 mg/day) was added. Muscle weakness and neurological abnormalities as well as abnormal laboratory findings gradually improved over the following two months. We conclude that the treatment with CPM combined with PSL may be useful, when neurological manifestations of MCTD are serious and resistant to conventional therapy.

Objective: To determine whether intravenous cyclophosphamide pulse therapy (IVCY) is effective for treating patients with diffuse proliferative lupus nephritis (DPLN) who were 1) refractory to methylprednisolone pulse therapy (MP) or 2) could not be treated with MP because of severe diabetes or steroid induced psychosis.

Methods: Seven patients with biopsy proven DPLN were studied after informed consent. Five of them received IVCY after a failure to achieve renal remission with at least 2 cycles of MP therapy. Of the other 2 patients, one had severe diabetes and the other a history of steroid induced psychosis. Bolus therapy with cyclophosphamide (0.5 g/m2 body surface area) was given once a month for 6 consecutive months and then once every 3 months for a total treatment period of 1 year. All patients were given oral prednisone, 0.5 mg/kg per day. The prednisone dose was tapered to the minimal dose required for controlling the disease. After 1 year, the renal status of the patients were evaluated.

Results: At 1 year, 4 of the 7 patients achieved substantial improvement. Although the other 3 patients did not satisfy the definition of substantial improvement, none of them had progressive disease. Adverse events were mild and did not require any treatment, with 2 cases of leukocytopenia without fever or major infection. No cases of hemorrhagic cystitis or amenorrhea were observed.

Conclusions: IVCY was 1) effective in the treatment of DPLN which was refractory to MP and 2) relatively safe with minimal side effects.

Autoimmune diseases such as systemic lupus erythematosus (SLE) are known to be causative disorders of reactive hemophagocytic syndrome (HPS). We recently encountered a case of HPS associated with the presence of antiphospholipid antibodies (aPL). This patient showed severe thrombocytopenia (0.2 x 10(4)/microliter) and moderate anemia (Hb; 7.6 g/dl). Bone marrow smears showed normal cellularity and an increase in mature-looking histiocytes scattered among the hematopoietic cells, which accounted for approximately 3% of all nucleated cells and were distributed unevenly. These cells showed marked phagocytosis of hematopoietic cells, including megakaryocytes, erythroblasts, and a few neutrophils. In this patient, there is no possible causative factor of HPS (such as viral infection, lymphoma, and systemic lupus erythematosus) except the presence of aPL. There have been no previously reported cases describing the relationship between aPL and HPS. This case indicate that attention should be given to the possibility that certain patients with aPL-associated cytopenia may display accompanying intramedullary hemophagocytic phenomena.

We report here two Japanese cases of rheumatoid arthritis (RA) associated with IgA [symbol: see text]-type multiple myeloma (MM). Case 1. The patient was a 68-year-old man with eight-years history of RA. The M-proteinemia (IgA 2838 mg/dl) in laboratory findings suggested a complication of MM which had been noticed since four years ago. On May 1997, he was referred and admitted to our hospital because of cough, right chest pain and dyspnea. Serum immunoelectrophoresis showed monoclonal IgA[symbol: see text]-type light chain. Bone marrow aspirate contained 6.5% atypical plasma cells. The X-ray findings revealed radiolucent myelomatous foci in the skull. From these findings, IgA[symbol: see text]-type MM was diagnosed. His condition was recovered by administration of antibiotics for bacterial pleuritis. Case 2. The patient was a 75-year-old woman with twelve-years history of RA. The laboratory findings of M-proteinemia (IgA 1215 mg/dl) with the decrease of other serum immunoglobulin level (IgG 611 mg/dl, IgM 60 mg/dl) and monoclonal IgA[symbol: see text]-type light chain in serum immunoelectrophoresis suggested MM four years ago. Bone marrow aspirate contained 5% plasma cells. From these findings, IgA[symbol: see text]-type MM was diagnosed. In the review of reported Japanese cases of RA associated with MM, it might be characteristic that IgA type MM was found more frequently in RA patients than other immunoglobulin types.

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.