Ryumachi. [Rheumatism]最新文献

We investigated clinical features of patients with osteoarthritic knees followed by development of rheumatoid arthritis (RA) after several year's interval. The subjects were 16 knees of 8 patients with osteoarthritis (OA) including one man and 7 women. The mean age at development of OA knee was 62.8 years (range; 45-73). The mean age at later development of RA was 66.0 years (range; 52-79). The mean follow-up period was 96.4 months (range; 28-191). We evaluated clinical features using the 1987 revised Criteria of the American College of Rheumatology (ACR), laboratory dates including RF, CRP, ESR, the number of joints with RA, and femorotibial angle (FTA). The mean number of features of patients which was fulfilled with the ACR criteria was 3.3 +/- 1.6 at the onset of RA. Only four patients were seropositive through the total follow-up period. The serum level of RF, CRP, and ESR were reduced at the follow-up period. The mean number of the joints involved in RA was 11.0 +/- 5.1 (range; 4-22) and wrist and shoulder joints were involved more frequently than other joints except knees. High tibial osteotomy (HTO) was performed on 5 knees of 3 patients and the mean degree of FTA was 168.8 +/- 1.9 degrees just after surgery. However, 36 months after development of RA, joint destruction and valgus deformity occurred on 3 knees and the mean degree of FTA of 5 knees was ended up to 159.6 +/- 11.3 degrees. Our experiences suggested that RF, CRP, ESR and lesions of other joints should be carefully evaluated in the OA patients with seropositivity or knee hydrarthrosis and that histological analysis for synovium should be assessed by the biopsy at time of HTO or arthroscopic surgery to improve accuracy of diagnosis.

A 19-year-old woman had been treated for bronchiectasis since she was born. In October 1995, she was diagnosed as Henoch-Schönlein purpura (HSP) and HSP nephritis with the findings as follows; palpable petechial rash of legs, abdominal pain, arthralgias, and proteinuria. The administration of oral prednisolone was started, the clinical symptoms except for proteinuria was disappeared. However, nephrotic syndrome was continued despite the therapy of intravenous methylprednisolone pulse, various immunosuppressive drugs and warfarin. In February 1998, she was admitted to our hospital because of pneumonia. Several days later, her pneumonia improved on treatment with antibiotics, but she suddenly developed transient cortical blindness and acute renal failure. A provisional diagnosis of hypertensive encephalopathy and cerebral edema related to vasculitis was made, and she was treated with nifedipine, Glycelo, and high dose immunoglobulin. After the treatment, her vision and renal function had improved. She is a rare case associated with transient cortical blindness, bronchiectasis, and HSP.

A 39-year-old man, who had been treated with Etretinate for common wart since he was 29 years old, was admitted to Taga General Hospital complaining of gradually deteriorating lumbago and bilateral hip joints pain in September, 1996. His lower vertebrae and bilateral hip joints showed abnormal ossification on X-ray. The bone scintigraphy indicated the existence of sacroiliitis. His platelet counts were fluctuating between 8 x 10(4) and 9 x 10(4)/mm3. During the follow-up in our out-patient clinic, he was suddenly suffered from severe abdominal pain in August, 1997 and admitted to our hospital. An emergency operation revealed multiple ulcers of his ileum with several perforations. Histological findings of the specimen of the ileum showed simple ulcer. After the operation, he had oral and genital ulcers. He did not have any signs or symptoms of ocular involvement. He was diagnosed as intestinal Behçet's disease. Because he showed gradually pancytopenia for several months after the operation, bone marrow aspiration was performed and a diagnosis of refractory anemia, a type of myelodysplastic syndrome (MDS), with trisomy of chromosome 8 was made. Abnormal ossification of his vertebrae and hip joints were considered to be related to Behçet's disease because an coexistence with sacroiliitis. On the other hand, there is no denying the effects of orally Etretinate administration. Several cases have been reported the association of MDS with Behçet's disease. In this case, the existence of MDS or various symptoms in Behçet's disease became apparent after the perforation of ileum ulcer. This paper discusses possible etiology of the relation between Behçet's disease and MDS, or the characteristic clinical course in this case.

Rheumatoid vasculitis is a relatively uncommon complication of rheumatoid arthritis (RA). It shows wide variety of extra-articular features including skin and neurologic involvement, but rarely shows gastrointestinal involvement. We describe a 79-old-man of rheumatoid vasculitis manifesting as small bowel multiple ulcerations accompanied by perforation of the descending colon diverticulum. The patient was admitted to our hospital with rectal bleeding and severe anemia. He had had rheumatoid arthritis for more than 10 years with treatment of non-steroidal anti-inflammatory drugs (NSAIDs) and low-dose prednisone. Upper endoscopy or colonoscopy revealed no evidence of bleeding. Suddenly he developed cramping abdominal pain, and emergency operation was performed. Segments of the descending colon and small bowel were resected to reveal perforation of the descending diverticulum and severe multiple ulcer of small bowel. The pathological examination showed multiple ulcerations of the small intestine was caused by vasculitis.

We report a case of Sjögren's syndrome (SS) and systemic sclerosis (SSc) complicated with acute pancreatitis. A 51-year-old woman had been diagnosed as SS in 1973. She noticed Raynaud's phenomenon in 1977. In 1988, interstitial pneumonia (IP) was pointed out and she was treated with methylprednisolone (mPSL) pulse therapy. Prednisolone (PSL) was gradually tapered to 3-5 mg daily and she visited our outpatient clinic in 1995. On her first admission to our hospital in 1996, she showed xerostomia, keratoconjunctivitis sicca, sclerotic skin changes of her distal extremities and face, thickening of her sublinguinal frenulum, and regurgitative esophagitis. She was positive with anti-SS-A and SS-B antibodies. She was diagnosed as SS and SSc. Radiographic and laboratory data also established the diagnosis of inactive IP, renal tubular acidosis (RTA) and chronic renal failure (CRF). In April 30th 1997, she was admitted to our hospital again with complaints of dyspnea, dysesthesia, epigastralgia and petechia. Active IP and mononeuritis multiplex were diagnosed, and petechia was considered to be associated with vasculopathy. Her serum amylase level was 891 mU/ml on admission and spontaneously increased to 2440 mU/ml on May 12th along with increase of fibrinogen degradation product, D-dimer and alpha 2 plasmin-plasmin inhibitor complex levels. Ultrasonography depicted swelling of her pancreatic head and the diagnosis of acute pancreatitis was made. She was treated with protease inhibitors and intravenous hyperalimentation for acute pancreatitis. mPSL pulse therapy (500 mg/day for 3 days) was instituted for IP and mononeuritis multiplex on May 22, followed by 50 mg of daily PSL. While IP and mononeuritis multiplex gradually improved by the high-dose steroid therapy, serum amylase level raised to more than 4293 mU/ml, suggesting the modification of pancreatitis by the treatment with steroid. Since she did not respond to the conservative therapy for acute pancreatitis, she was treated with plasmapheresis, which turned out to be very effective. However, she was suffered from fungal pneumonia and died of respiratory failure. As far as we know, only three cases of SS with acute pancreatitis have been reported so far. The immunopathological mechanisms of development of acute pancreatitis in our case, especially focusing on the significance of microvasculopathy and hypercoagulability, were discussed.

A fifteen-year-old boy was admitted to our hospital because of lower abdominal pain, watery diarrhea and mucobloody stool. Two years before admission, he was diagnosed to have Still's disease presenting with polyarthritis, sore throat, remittent fever and typical skin rash. He had been treated with non-steroidal anti-inflammatory agents, oral prednisolone and low-dose methotrexate. Although he was almost free of symptoms during the next two years, serum C-reactive protein (CRP) levels continued to be elevated moderately. He began to complain of lower abdominal pain and loose stool in May 1997 and came down with mucous-bloody diarrhea in June. Laboratory data on admission showed an elevated level of serum CRP (13.9 mg/dl). The biopsy of the stomach, ileum, sigmoid colon and rectum revealed the deposition of amyloid protein of AA type, which confirmed the diagnosis of secondary amyloidosis. The dose of prednisolone was increased and dimethyl sulfoxide per os or rectum was instituted, which improved his gastro-intestinal symptoms to some extent. However, fever, arthritis and diarrhea recurred along with tapered prednisolone dosage. In addition to gastro-intestinal symptoms, arrhythmia and proteinuria appeared. These symptoms were considered to reflect general deposition of amyloid in his body. He is now on immunosuppressive agent and high-dose prednisolone. Several studies report the higher frequency of gamma-allele of SAA 1 gene in the cases of rheumatoid arthritis with AA-amyloidosis than in those without. In the patient presented here, molecular biological analysis revealed that his SAA 1 gene was composed of beta- and gamma-allele. The presence of gamma-allele in his SAA 1 gene might be one of the factors that predisposed him for generalized deposition of amyloid protein in such a short period of time.