Wegener's granulomatosis (WG) is highly correlated with cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA). Patients with rheumatoid arthritis (RA) rarely contract WG. Although several concurrent cases have been reported overseas, there are no known cases in Korea. Here we report a unique case of RA with atypical WG testing positive for perinuclear ANCA (p-ANCA) and negative for anti-myeloperoxidase (MPO) antibodies. The 62-yr-old female patient presented with multiple joint pain and showed typical blood test results for RA, i.e., an elevated erythrocyte sedimentation rate and C-reactive protein concentration, and positive for rheumatoid factor and anti-cyclic citrullinated peptide antibodies. RA was clear based on a total score of 10 when applying the classification criteria developed by the American College of Rheumatology/European League Against Rheumatism (2010). In an autoimmune target test, speckled and skeleton patterns were observed. In an ANCA test, p-ANCA was observed (titer, 1:2,560), and tests for anti-proteinase 3 (PR3) and anti-MPO antibodies were negative. After admission, multiple nodules were detected on a chest X-ray and a computed tomography scan. We suspected that she had rheumatic nodules or vasculitis and performed an open lung biopsy. We detected necrotic granulomatous vasculitis, classified as WG, thus leading to WG diagnosis. In conclusion, WG was diagnosed in an RA patient who was negative for c-ANCA (negative PR3) and positive for p-ANCA (negative anti-MPO), and this peculiar finding is likely to improve diagnosis in cases of RA with atypical WG.
Antibodies ; Antibodies, Antineutrophil Cytoplasmic ; Arthralgia ; Arthritis, Rheumatoid* ; Biopsy ; Blood Sedimentation ; C-Reactive Protein ; Classification ; Cytoplasm ; Diagnosis ; Female ; Hematologic Tests ; Humans ; Korea ; Lung ; Rheumatic Diseases ; Rheumatic Nodule ; Rheumatoid Factor ; Skeleton ; Thorax ; Vasculitis ; Wegener Granulomatosis*

The myelodysplastic syndrome (MDS) is a heterogeneous group of refractory anemia resulting from a clonal abnormality in the pleuripotent stem cell. A variety of immunologic abnormalities have been reported in patients with MDS, and various rheumatic diseases, including rheumatoid arthritis, lupus, Sjogren? syndrome, vasculitis, have been reported in association with MDS. Inflammatory arthritis is a rare complication of MDS. Here we report a patient with refractory anemia with excess blasts with unusual manifestation of inflammatory arthritis, with a review of the literature.
Anemia, Refractory ; Arthritis* ; Arthritis, Rheumatoid ; Humans ; Myelodysplastic Syndromes* ; Rheumatic Diseases ; Stem Cells ; Vasculitis

Relapsing polychondritis is a rare condition of unknown etiology in which recurrent episodes of inflammation are followed by the destruction of cartilaginous structures, which predominantly include the nose, ears, and tracheobronchial tree. Although many autoimmune disorders, such as systemic vasculitis, rheumatoid arthritis, lupus erythematosus, Sjogren's syndrome, and Behcet's disease, are commonly found in patients with relapsing polychondritis, ankylosing spondylitis is rarely associated with this disease. We report a case of relapsing polychondritis with ankylosing spondylitis in the same patient.
Arthritis, Rheumatoid ; Ear ; Humans ; Inflammation ; Nose ; Polychondritis, Relapsing ; Sjogren's Syndrome ; Spondylitis, Ankylosing ; Systemic Vasculitis

Bronchiolitis obliterans organizing pneumonia(BOOP) is a pathologic entity characterized by the formation of plugs of fibrous tissue in bronchioles and alveolar ducts. It had been described in association with several connective tissue diseases, i.e. systemic lupus erythematosus, Behcet's disease, polymyositis-dermatomyositis, polymyalgia rheumatica and Sjogren's syndrome. Recently, there were few additional reports on BOOP, anteceded by rheumatoid arthritis. We had experienced a case of 60 years old female patient who had been admitted for acute respiratory difficulty and abnormal chest roentgenographic findings. She was diagnosed as having rhematoid arthritis 20 years ago. On the course of our treatment, she did not respond to antibiotics. Moreover, no microorganism grew in the sputum and blood. Thus, the bronchoscopic biopsy was done. The patient showed a dramatic response to steroid therapy, and the pathologic findings of the bronchoscopic biopsy confirmed as BOOP. To our knowledge, this is the first reported case of BOOP anteceded by rheumatoid arthritis in Korea.
Anti-Bacterial Agents ; Arthritis ; Arthritis, Rheumatoid* ; Biopsy ; Bronchioles ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Connective Tissue Diseases ; Cryptogenic Organizing Pneumonia* ; Dermatomyositis ; Female ; Humans ; Korea ; Lupus Erythematosus, Systemic ; Polymyalgia Rheumatica ; Sjogren's Syndrome ; Sputum ; Thorax

Arthritis, Juvenile/complications* ; Humans ; Rheumatoid Nodule

Chronic arthritis, the most common chronic rheumatic disease of childhood, is one of the most frequent chronic illness of children and an important cause of short and long term disability. It is not a single disease, but a group of related, genetically heterogeneous, phenotypically diverse immunoinflammatory disorders that affect the joints and other structures, possibly activated by contact with external antigens. Since its introduction in 1994, the term Juvenile Idiopathic Arthritis (JIA) has largely supplanted the terms Juvenile Chronic Arthritis and Juvenile Rheumatoid Arthritis. However, it is necessary to understand the older classifications in order to interpret the literature on the subject. Each subtype of JIA is to provide a general introduction.
Arthritis ; Arthritis, Juvenile Rheumatoid ; Child ; Chronic Disease ; Humans ; Joints ; Rheumatic Diseases

Adult onset Still's disease (AOSD) is a rare systemic inflammatory disease that is basically an adult onset of juvenile rheumatoid arthritis, and the manifestations include fever, rash, arthritis and other organ involvement. The typical skin rash of AOSD is evanescent, erythematous maculopapular lesions. We report here on a rare case of AOSD in a 29-year-old female patient who presented with high fever, erythma multiforme-like skin eruption, arthritis and sore throat. She was diagnosed with AOSD according to the diagnostic criteria of Yamaguchi.
Adult ; Arthritis ; Arthritis, Juvenile Rheumatoid ; Erythema ; Erythema Multiforme ; Exanthema ; Female ; Fever ; Humans ; Pharyngitis ; Skin ; Still's Disease, Adult-Onset

No abstract available.
Arthritis, Rheumatoid* ; Vasculitis*

BACKGROUND: Anti-streptolysin O (ASO) test is usually used to diagnose group A streptococcal infection-related diseases, such as rheumatic fever, reactive arthritis, and various infectious diseases. Despite the recent declining incidence of these diseases, ASO test is still frequently performed as a screening test to diagnose rheumatic diseases. This study re-evaluated the clinical usefulness of ASO test in systemic rheumatic diseases (SRD). METHODS: ASO tests was performed in 825 patients between April and October in 2010. ASO levels were compared between SRD and non-SRD groups of patients. The results of ASO, C-reactive protein (CRP), and rheumatoid factor (RF) were compared among 6 subgroups of SRD: rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis, Behcet disease, Sjogren's syndrome and others. RESULTS: Positive results in ASO test (>200 IU/mL) were observed in 15.3% (126/825) of the patients tested. None of the ASO positive patients was, however, diagnosed with rheumatic fever or reactive arthritis. There were no statistically significant differences in the mean value (P=0.688) or positive rate (P=0.835) of ASO test between SRD and non-SRD groups. Positive rates of ASO test were also not statistically significant different among six subgroups of SRD patients (all P>0.05), whereas those of CRP and RF tests were significantly different. CONCLUSIONS: The usefulness of ASO test is very low for diagnosing SRD, although it is frequently carried out as a screening test. We suggest that ASO test must be performed selectively when diseases from group A streptococcal infection are suspected.
Arthritis, Reactive ; Arthritis, Rheumatoid ; Behcet Syndrome ; C-Reactive Protein ; Communicable Diseases ; Humans ; Incidence ; Lupus Erythematosus, Systemic ; Mass Screening ; Rheumatic Diseases ; Rheumatic Fever ; Rheumatoid Factor ; Sjogren's Syndrome ; Spondylitis, Ankylosing ; Streptococcal Infections

OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA) is known to be associated with vasculitides. But recently ANCA has been reported in some chronic inflammatory diseases regardless of vasculitic condition. Although the incidence of vasculitis was low in Korea, we occasionally encounter patients with positive ANCA. And it was doubtful whether ANCA positivity of reports, in time that ANCA test was introduced in Korea, was true. So this study aimed to reevaluate the clinical utility of ANCA with the modern stabilized substrate. METHODS: 162 patients with positive ANCA from Jan. 2002 to Jan. 2003 were investigated. ANCA was tested by indirect immunofluorescence assay using ALCA kit (ImmunoThink(R), Korea). RESULTS: Of 162 patients, 158 were P-ANCA positive, 2 were C-ANCA positive and 2 were atypical C-ANCA positive. Most of the ANCA positive patients had chronic inflammatory diseases-129 patients (79.6%) with rheumatoid arthritis (RA), 4 (2.5%) with lupus and 4 (2.5%) with ulcerative colitis. Only 3 patients (1.9%) had vasculitides and the remaining seventeen patients had other diseases. Among the RA patients with ANCA, 27.9% were seronegative RA. CONCLUSIONS: In order to increase the clinical applicability of ANCA test in diagnosing vasculitides, it is essential to decrease the false positivity by using stabilized substrate and by adapting professional interpretation from experts. And our results suggest that ANCA test can be useful for diagnosing chronic inflammatory diseases such as rheumatic diseases (especially RA) along with vasculitides in Korea. Further study will be needed for the diagnostic utility of ANCA in RA.
Antibodies, Antineutrophil Cytoplasmic* ; Arthritis, Rheumatoid ; Colitis, Ulcerative ; Fluorescent Antibody Technique, Indirect ; Humans ; Incidence ; Korea ; Rheumatic Diseases ; Vasculitis