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

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

OBJECTIVE: To investigate the frequency of autonomic nervous dysfunction and assess the factors associated with autonomic nervous dysfunction in patients with rheumatoid arthritis (RA). METHODS: Three battery of cardiovascular autonomic nervous function tests (CAN test);heart rate response to Valsalva maneuver,deep breathing and standing up,were performed in 68 patients who met the 1987 American Rheumatism Association revised criteria for RA and 58 healthy controls.Autonomic nervous dysfunction was defined as 2 of 3 CAN tests were abnormal. Clinical and radiographic variables such as age,sex,disease duration,erythrocyte sedimentaton rate (ESR)and bone destruction,were analyzed in patients with or without autonomic nervous dysfunction. RESULTS: In patients with RA,the frequency of autonomic nervous dysfunction was higher than healthy controls (50.8%in RA vs 15.5%in healthy controls,p<0.05).Age,sex,disease duration,ESR and bone destruction were not associated with autonomic nervous dysfunction in patients with RA CONCLUSIONS: This results suggest that autonomic nervous dysfunction may be a significant feature in patients with RA.
Arthritis, Rheumatoid* ; Humans ; Respiration ; Rheumatic Diseases

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

BACKGROUND: The American College of Rheumatology/European League against Rheumatism classification criteria for rheumatoid arthritis (ACR/EULAR criteria) include the rheumatoid factor (RF) test and the anti-citrullinated peptide/protein antibody (ACPA) test as serologic makers for rheumatoid arthritis. Antiperinuclear factor (APF) test, an originator of ACPA, is highly specific for rheumatoid arthritis and can be detected in RF or anti-cyclic citrullinated peptide (anti-CCP) negative rheumatoid arthritis, but it is not included in the serologic criterion of ACR/EULAR criteria. In this study, we investigated the way for applying the APF test to ACR/EULAR criteria. METHODS: We analyzed clinical symptoms and laboratory findings of 53 patients who were suspected having rheumatoid arthritis. All patients were negative for the RF and anti-CCP and positive for APF. We classified these patients into 4 groups according to the fluorescence intensity of APF test, and gave 1-4 points for APF positivity. The proportion of patients who scored 6 or greater in ACR/EULAR criteria in relation to APF scores was evaluated. RESULTS: Median scores of ACR/EULAR criteria showed a tendency to increase as the level of fluorescence intensity of APF rises, but ACR/EULAR scores of 4 groups were not different significantly from each other (P>0.05). The proportion of patients who scored 6 or greater in ACR/EULAR criteria were 39.6% and 77.4%, when scores of APF positivity were 2 and 3 points, respectively. CONCLUSIONS: We think it is reasonable to include APF test in the ACPA of ACR/EULAR criteria and give 3 points for APF positivity, regardless of its fluorescence intensity.
Antibodies, Antinuclear ; Arthritis, Rheumatoid ; Fluorescence ; Humans ; Rheumatic Diseases ; Rheumatoid Factor

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome is a rare condition that occurs in elderly individuals. It is characterized by sudden onset of bilateral symmetrical distal tenosynovitis that accompanied by obvious swelling of the hand with pitting edema and absence of rheumatoid factor (RF). This disease entity sometimes presented as overlap syndrome with other rheumatic diseases and needed to be differentiated from those. However, if the diagnosis is confirmed, the response to steroid is good. The purpose of this report is to describe the case of RS3PE syndrome presented with bilateral hand dorsum edema in a middle-aged woman.
Aged ; Diagnosis ; Edema ; Female ; Hand ; Humans ; Polymyalgia Rheumatica ; Rheumatic Diseases ; Rheumatoid Factor ; Synovitis ; Tenosynovitis

Juvenile idiopathic arthritis (JIA) is a broad spectrum of disease defined by the presence of arthritis of unknown etiology, lasting more than six weeks duration, and occurring in children less than 16 years of age. JIA encompasses several disease categories, each with distinct clinical manifestations, laboratory findings, genetic backgrounds, and pathogenesis. JIA is classified into seven subtypes by the International League of Associations for Rheumatology: systemic, oligoarticular, polyarticular with and without rheumatoid factor, enthesitis-related arthritis, psoriatic arthritis, and undifferentiated arthritis. Diagnosis of the precise subtype is an important requirement for management and research. JIA is a common chronic rheumatic disease in children and is an important cause of acute and chronic disability. Arthritis or arthritis-like symptoms may be present in many other conditions. Therefore, it is important to consider differential diagnoses for JIA that include infections, other connective tissue diseases, and malignancies. Leukemia and septic arthritis are the most important diseases that can be mistaken for JIA. The aim of this review is to provide a summary of the subtypes and differential diagnoses of JIA.
Arthritis ; Arthritis, Infectious ; Arthritis, Juvenile* ; Arthritis, Psoriatic ; Child ; Connective Tissue Diseases ; Diagnosis ; Diagnosis, Differential* ; Genetic Background ; Humans ; Leukemia ; Rheumatic Diseases ; Rheumatoid Factor ; Rheumatology

A 50-year-old woman, who had been treated for rheumatoid arthritis (RA) over a 10-year period, suddenly presented with monocular vision loss while the RA had a stable course over many years. She was diagnosed with central retinal artery occlusion (CRAO) based on ophthalmologic examinations including optical coherence tomography and fluorescein angiography. There was no evidence of atherosclerosis, infection, and malignancy that can cause CRAO. Considering the association between CRAO and other rheumatic diseases, such as systemic vasculitis and systemic lupus erythematous in previous reports, it was presumed that her RA might have contributed to the development of CRAO. Although cases of CRAO in patients with RA are extremely rare, these findings suggest that physicians need to be aware of the possibility of CRAO in patients with RA who experience decreased visual acuity.
Arthritis, Rheumatoid* ; Atherosclerosis ; Female ; Fluorescein Angiography ; Humans ; Middle Aged ; Retinal Artery Occlusion* ; Retinal Artery* ; Rheumatic Diseases ; Systemic Vasculitis ; Tomography, Optical Coherence ; Vision, Monocular ; Visual Acuity