ABSTRACT
The camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) is characterized by congenital or early-onset camptodactyly, childhood-onset noninflammatory arthropathy associated with synovial hyperplasia. Some patients have pro-gressive coxa vara deformity and/or noninflammatory pericardial effusion. CACP is inherited as an autosomal recessive mode and the disease gene is assigned to a 1.9-cM interval on human chromosome 1q25-31. We describe a 10-yr-old boy who has typical features of CACP without familial association.
Subject(s)
Adolescent , Humans , Male , Fingers/abnormalities , Hip Joint/abnormalities , Joint Diseases/congenital , Pericarditis/congenital , Syndrome , Toes/abnormalitiesABSTRACT
OBJECTIVES: In the connective tissue disease patients, esophageal dysfunction is often closely associated with the presence of Raynaud's phenomenon. But there are no previous reports concerning the values of esophageal manometry in the connective tissue disease with Raynaud s phenomenon in Korea. Therefore, we performed this study to evaluate esophageal function in connective tissue disease with Raynaud' s phenomenon. METHODS: Total 86 subjects were employed in this study including 30 normal control group, 14 mixed connective tissue disease(MCTD), 21 systemic sclerosis, 16 systemic lupus erythematosus(SLE), and 5 Raynaud s phenomenon only. In each subject, esophageal manometric study was performed with lower compliance capillary infusion system. RESULTS: The mean age(+SD) of patients and controls were as follows : MCTD 34.1(+8.9), systemic sclerosis 44.9(+9. 3), SLE 32. 1(+7.9), and normal controls 31. 9 (+ 5. 3). All patients with MCTD and systemic sclerosis had Raynaud s phenomenon. Twelve out of 14 patients with MCTD, 17 out of 21 patients with systemic sclerosis, were abnormal in esophageal manometry. Nine out of 16 SLE had Raynaud s phenomenon. Among 9 SLE with Raynaud s phenomenon, 5 patients(55.5%) were abnormal in esophageal manometry, and among 7 SLE without Raynaud's phenomenon, 2 patients (28. 6%) were abnormal in esophageal manometry. Among 5 patients with Raynaud's phenomenon, 3 patients were abnormal in esophageal manometry. Abnormal esophageal manometry finding is more common in the connective tissue disease patients with Raynaud's phenomenon compared with the patients without Raynaud's phenomenon (P=O. 0219). CONCLUSIONS: Abnormal esophageal manometry finding is more common in the connective tissue disease patients with Raynauds phenomenon compared with the patients without Raynaud s phenomenon.
Subject(s)
Humans , Capillaries , Compliance , Connective Tissue , Connective Tissue Diseases , Korea , Manometry , Mixed Connective Tissue Disease , Scleroderma, SystemicABSTRACT
OBJECTIVE: This study was undertaken to review the disease course, clinical and laboratory manifestations, prognosis and treatment of adult onset Still s disease (AOSD) in Korea. METHODS: Thirty-two patients with AOSD were enrolled from 1986 to 1997 in Hanyang University Hospital. Diagnosis of AOSD was based on the criteria proposed by Yamaguchi. We classified the disease course into self-limited, inter mittent, or chronic disease course. RESULTS: Twenty-four (75%) patients were female. Skin rash occurred in 28 (88%) patients, lymphadenopathy in 8 (25%), hepatomegaly in 4 (13%), and pericarditis in 2 (6%) out of 32 patients. The most commonly affected joints were knee joints (88%). Elevated LDH was seen in 18 (60%) patients and decreased CK in 17 (61%) patients. Rheumatoid factor was detected in 4 (13%) patients and ANA in 12 (38%) patients. Anemia (Hb < 10 g/dL) was seen in 13 (41%) patients and hypoalbuminemia (<3. 5 g/dL) in 14 (52%) patients. Elevated ferritin (300 ng/mL) level was seen in 23 (79%) patients. Twenty-five (78%) patients had elevated serum transaminase. Bone marrow studies were performed in 16 patients. Nine out of 16 patients showed hyperplasia of the myeloid series and 2 patients displayed the features of a hemophagocytic syndrome. The mean duration of follow up of 32 patients was 32 months (range 3- 108). Eight (27%) patients had a self-limited, 9 (30%) an intermittent, and 13 (43%) a chronic disease course. The hypoalbuminemia was significantly associated with an "intermittent or chronic disease group" (p<0. 05). Thirty-two patients received systemic corticosteroids and 21 patients received single or combination of disease modifying antirheumatic drugs. CONCLUSION: We found that hypoalbuminemia at presentation was significantly associated with an unfavorable outcome, intermittent or chronic disease group. The clinical manifestations and disease course of AOSD in Korea were similar to those previously reported in other countries except significantly lower incidence of lymphadenopathy, hepatomegaly, and pericarditis.
Subject(s)
Adult , Female , Humans , Adrenal Cortex Hormones , Anemia , Antirheumatic Agents , Bone Marrow , Chronic Disease , Diagnosis , Exanthema , Ferritins , Follow-Up Studies , Hepatomegaly , Hyperplasia , Hypoalbuminemia , Incidence , Joints , Knee Joint , Korea , Lymphatic Diseases , Lymphohistiocytosis, Hemophagocytic , Pericarditis , Prognosis , Rheumatoid Factor , Still's Disease, Adult-OnsetABSTRACT
Rice bodies are numerous small fibrinous, cartilaginous-like materials which were first described in tuberculous joints. Rice bodies are common findings in joints afflicted with rheumatoid arthritis or other seronegative arthropathy. But less commonly, those can be seen in periarticular bursae or at the sites of tendon or ligament insertion. Rice bodies, thought to be a nonspecific response to synovial inflammation, probably evolve from ischemia in a proliferative synovium. Some of these bodies contain a core of collagen with a mantle of fibrin, others contain only fibrin. Subacromial bursa are sometimes involved in the patients with rheumatoid arthritis and can reach impressive dimensions before becoming clinically detectable because significant constrain is lacking. Subacromial arthrography or magnetic resonance imaging accurately delineated the existence of cartilaginous loose bodies before surgical exision. We experienced a man with rheumatoid arthritis who had massive subacromial bursitis with rice bodies. His shoulder had been swollen since 5 years ago. He felt no pain and had only mild limitation of motion. Subacromial arthrography or magnetic resonance imaging of right shoulder showed multiple rice bodies in enlarged subacromial bursa. Operation finding showed a large encapsulated mass in the subacromial bursa, and hundreds of fibrinous rice bodies were revealed, which were resected. We report this patient with a review of literatures.
Subject(s)
Humans , Arthritis, Rheumatoid , Arthrography , Bursitis , Collagen , Fibrin , Inflammation , Ischemia , Joints , Ligaments , Magnetic Resonance Imaging , Shoulder , Synovial Membrane , TendonsABSTRACT
No abstract available.
Subject(s)
Antibodies, Antiphospholipid , Antiphospholipid Syndrome , Mixed Connective Tissue DiseaseABSTRACT
OBJECTIVE: To investigate the clinical characteristics of Korean JRA and the possible profiles related to prognosis and to compare these data to pre-existing occidental reports. METHODS: 140 cases of JRA patients were randomly selected from 1986 through 1995. Juvenile ankylosing spondylitis was excluded with the aid of modified New York criteria for ankylosing spondylitis. We used the classification of progression of rheumatoid arthritis (radiological stage III+IV / I+II+III+IV) as a index of prognosis. The study factors were sex, age, type, affected joints, degree of destruction of joint, anti-nuclear antibody (ANA), rheumatoid factor (RF), HLA-B27 and extra-articular manifestations. Chi-square test, One-way ANOVA test, and Pearson' s correlation coefficient were used as statistical methods. RESULTS: Our study show results as follows : (1) Systemic onset type (ll%) { polyarthritis subset (66%), oligoarthritis subset (34%) }, oligoarthritis onset type (62%) { sero (-) -HLA-B27 (+) subset (66%) , RF (+) subset (20%), not otherwise classified subset (14%) ANA(+)-chronic uveitis subset(O%) }, polyarthritis onset type (27%) { RF(+) subset (66%), not otherwise classified subset (34%) }, (2) Sex ratio was M:F=1.8:l. (3) Age of onset was 11+3.6 years. (4) Affected joints were knee, ankle, hand in decreasing order of frequency. (5) Patients show seropositivity of RF in 31%, HLA-B27 in 54%, ANA in 8%. (6) Chronic uveitis was observed in 7 cases (5%) (all oligoarthritis onset type, M:F=6:1, age of onset 11+4.3 years, ANA (0%), HLA-B27 (86%) , 2 cases leading to blindness. (7) Destructive bone change was significantly more prominent in female (p<0.01) , RF(+) (p<0.01), HLA-B27(-) (p<0.01) , polyarthritis onset type (p=0.02). CONCLUSIONS: In this study, we obtained interesting results that are somewhat different from Occidental data in sex ratio (male dominance), age of onset (older age) , profiles of HLA-B27 (high positivity in spite of low HLA-B27 positivity in Korean (about 2. 3%) , low rate of destructive change) , ANA (low positivity, older age of onset) , chronic uveitis (low incidence, male dominance, older age of onset, high association with HLA-B27, not associated with ANA) . This study suggests possible racial difference in clinical features of JRA. But for prove of racial difference, further multi-center trial and large scale epidemiological study should be done.
Subject(s)
Female , Humans , Male , Age of Onset , Ankle , Antibodies, Antinuclear , Arthritis , Arthritis, Juvenile , Arthritis, Rheumatoid , Blindness , Classification , Epidemiologic Studies , Hand , HLA-B27 Antigen , Incidence , Joints , Knee , Prognosis , Rheumatoid Factor , Sex Ratio , Spondylitis, Ankylosing , UveitisABSTRACT
SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome designates a group of articular and osseous manifestations frequently combined with skin disorders. Its fundamental component is inflammatory, pseudoinfectious, and sterile osteitis. The anterior chest wall is the most frequent localization and all the components of this structure may be involved. Palmoplantar pustulosis, psoriasis, acne conglobata, acne ulcerans, acne fulminans, pyoderma gangrenosum can be associated with the characteristic bone lesions. We report two cases of SAPHO syndrome : A 40-year-old female presented with both buttock pain with hyperostosis, costochondritis, synovitis and pustulosis palmaris and a 23-year-old male presented with migrating polyarthritis with costochondritis, synovitis, acne, pustulosis.
Subject(s)
Adult , Female , Humans , Male , Young Adult , Acne Vulgaris , Acquired Hyperostosis Syndrome , Arthritis , Buttocks , Hyperostosis , Osteitis , Psoriasis , Pyoderma Gangrenosum , Skin , Synovitis , Thoracic WallABSTRACT
Intradural extramedullary spinal metastasis from systemic tumor is extremely rare but epidural extramedullary cord tumor metastasis from the lung is relatively common. A 57 year-old male patient was admitted to department of internal medicine and neurosurgery in Chonbuk National University Hospital because of coughing, low back pain radiate to the right great toe, and numbness of the right calf area. Spinal MRI scan revealed round oval shaped mass lesion on just below the level of the conus medullaris. Authors present the clinical, histologic, radiologic features of spinal intradural metastatic tumor and operative total removal followed by chemothrapy with an extensive review of literatures.