Rheumatoid arthritis (RA) is a systemic inflammatory disease caused by abundant inflammatory cytokines produced by lymphocytes, macrophages and fibroblast-like synoviocytes. RA presents many extraarticular manifestations like interstitial pneumonia (IP), amyloidosis, rheumatoid nodules and vasculitis. We are often confronted with difficulties in therapy for drug-resitance RA, drug-allergy RA and RA patients with complications such as IP or renal failure. Currently, over 3500 RA patients have received a treatment called leukocytapheresis (LCAP) in Japan. LCAP is considered a safe and effective therapy, but reports for LCAP are limited. To determine the efficacy and safety of LCAP for refractory RA, drug-resistance RA and malignant RA (MRA), we performed LCAP to 13 RA patients once a week for five weeks, and measured the effectiveness. At the point of 4 weeks after the last LCAP, ACR20 and ACR50 were 30.1% and 7.7%, respectively and the mean of ACR-N was 16.9%. One patient with neuropathy and reticular erythema completely recovered through LCAP. These symptoms did not relapse at 12 months post LCAP. Another patient having undergone hemodialysis (HD) was safely treated with LCAP following regular HD. Five patients with IP also received LCAP safely, although improvement of IP was not found. Adverse reaction to LCAP was limited to nausea and no severe side effects were found. LCAP is a safe and effective therapy for refractory RA, drug-resistance RA and MRA.

View full abstract

    Sjögren’s syndrome (SjS) is very rare in childhood. Secondary SjS followed by systemic lupus erythematosus sometimes occurs; however, this disease followed by juvenile idiopathic arthritis (JIA) has only rarely been reported. We encountered a young woman with juvenile SjS who developed rheumatoid arthritis (RA) at the age of 19 years, 9 years after the onset of SjS.
    At age 10, this patient visited our hospital with low-grade fever, erythema, lymphadenopathy, dryness of mouth, arthralgia, and general fatigue. Laboratory examinations showed elevated serum total protein, high serum IgG, positive autoantibodies such as antinuclear antibody, rheumatoid factor, anti-Ro/SS-A antibody, and anti-La/SS-B antibody. Histopathological and sialographic changes in salivary glands and hypofunction of the lacrimal glands were also revealed. We diagnosed the patient with primary SjS and treated her with predonisolone, mizoribin, and methotrexate thereafter.
    Nine years after the onset of SjS, the patient developed arthralgia and swelling of the finger joints, and arthritis extended to the joints of both hips, the right knee, and the right shoulder with morning stiffness. In laboratory findings, C-reactive protein, erythrocyte sedimentation rare, and serum matrix metalloproteinase (MMP)-3 were elevated. The diagnosis of RA is confirmed by those findings. She was considered to have secondary SjS with RA based on the course of diagnosis.
    In a juvenile SjS patient, the symptoms of RA morbidity may develop with delayed onset. In the patient diagnosed with JIA, they may have overlooked the morbidity of SjS.

View full abstract

    A 27-year old female, who had been diagnosed as mixed connective tissue disease (MCTD) due to Raynaud’s phenomenon, swollen hands, polyarthritis, myositis, generalized erythema and elevation of anti-U1 RNP antibody titer, was successfully treated with oral prednisolone, presented exacerbations with fever, lymphadenopathy, polyarthralgia and myalgia. In March 2006, she was admitted to our hospital because of fever, headache and syncope. Physical examination on admission revealed facial erythema, complete right hemiplegia and disturbance of consciousness (Glasgow Coma Scale 11). Laboratory examinations showed decreased serum complement level and elevation of serum anti-DNA antibody level. Cerebrospinal fluid (CSF) interleukin (IL)-6 was also markedly elevated. MRI scans revealed high intensity areas along the left ventricle on diffusion weighted images and FLAIR images. Two courses of methylprednisolone pulse therapy followed by 100 mg/day of prednisolone for 4 weeks improved all of her symptoms and laboratory findings along with the disappearance of high intensity areas on MRI. The cerebral reversible lesions in this patient might possibly be caused by vasculitis due to SLE.

View full abstract

    Multicentric reticulohistiocytosis (MRH) is a rare systemic disease of unknown etiology: it is characterized by two major symptoms―proliferative, destructive polyarthritis and nodular lesions with the proliferation of histiocytes of the skin and mucous membrane. We had an opportunity to conduct an autopsy on a 54-year-old woman with MRH who suddenly succumbed to multifocal myocardial infarction. The patient had developed polyarthralgia, muscle weaknessof the extremities and skin symptoms. Initially, the possibility of dermatomyositis was considered but she was non-responsive to prednisolone, while the cutaneous nodules at her elbow were enlarged and destructive arthritis progressed. In spite of implantation of a permanent pacemaker, she eventually succumbed to cardiac arrest. At autopsy, diffuse infiltration by swollen histiocytes was noted in the synovium, trachea, lungs, spleen, pancreas, esophagus, small intestine and urinary bladder. However, the cardiac muscle was totally free of cell infiltration. The major cause of death was hemorrhagic infarction of the ventricular septum. The literature yields only one description of MRH that was observed at autopsy. We believe that this experience is extremely rare.

View full abstract

    The patient was a 37-year-old female. An abdominal pain appeared in the latter part of March 2005, and she was first suspected as gastroenteritis and urinary tract infection. Early in April, an exanthema, similar to palpable purpura, exhibited on the cruris, and was diagnosed as Henoch-Schönlein purpura (HSP). Her gastro-intestinal symptoms exacerbated, and she was admitted to our hospital on April 22nd.Torous erythema and purpura appeared on the upper and lower abdomen, and both femorals. Laboratory examinations showed an increased WBC count, high levels of ESR and CRP. The upper endoscopy showed vasodilation and hyperlucency of the mucosa, and purpura like findings in the stomach. The vascular hyperlucency, the redness and vasodilation were observed by the colon endoscopy. Compatible findings with HSP were presented by skin biopsy, though not by the colon biopsy. We diagnosed the abdominal pain associated to HSP. After treatments with non-peroral, hemostyptic, vitamin C, and medicine for intestinal disorders and peptic ulcer, her symptoms improved, and did not worsen after the diet started. HSP is usually seen in children, and is accompanied with skin, joint, abdominal and renal symptoms. Cases with prior abdominal symptoms occur mostly to children, and are rare among adults in Japan.

View full abstract