No abstract available.
Adult ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*complications/diagnosis/drug therapy/immunology ; Antibodies, Antineutrophil Cytoplasmic/*blood ; Biomarkers/blood ; Biopsy ; Drug Therapy, Combination ; Fluorescent Antibody Technique ; Glomerulonephritis, IGA/*complications/diagnosis/drug therapy/immunology ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Myeloblastin/*immunology ; Treatment Outcome

OBJECTIVETo study the clinical significance of anti-endothelial cell antibodies (AECA) and anti-neutrophil cytoplasmic antibodies (ANCA) in Kawasaki disease (KD) and its complication of coronary arterial lesions (CAL).

METHODSForty-two children with KD, as well as 20 children with fever caused by respiratory infection (fever control group) and 15 children for selective operation (normal control group), were included in the study. Serum levels of AECA and ANCA were measured using enzyme-linked immunosorbent assay. Echocardiography was performed to evaluate CAL in KD patients.

RESULTSDuring the acute phase, the KD patients had significantly higher serum AECA and ANCA levels than the two control groups (P<0.01). The KD patients had reduced serum ANCA levels (P<0.01) in the remission phase, but they were still higher than those of the two control groups (P<0.05). Among KD patients, those with CAL had significantly higher ANCA levels than those without CAL (P<0.01) in the acute phase. There was a positive correlation between serum ANCA levels and the ratio of left coronary artery to aortic annular diameter in KD patients with CAL (r=0.88, P<0.01).

CONCLUSIONSAECA and ANCA may be involved in vasculitis and CAL among children with KD. Serum levels of AECA and ANCA may be used as indicators for the diagnosis of suspected KD cases in the acute phase. Elevated ANCA level has a certain predictive value for CAL.

Antibodies, Antineutrophil Cytoplasmic ; blood ; Autoantibodies ; blood ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; immunology ; Severity of Illness Index

BACKGROUND/AIMS: Churg-Strauss syndrome (CSS) is a rare systemic necrotizing small-vessel vasculitis, with accompanying bronchial asthma, eosinophilia, and eosinophilic infiltration of various tissues. The purposes of our study were to characterize the clinical features of CSS and to identify factors associated with CSS prognosis in Koreans. METHODS: Medical records were reviewed retrospectively for all physician-diagnosed CSS patients in the Seoul National University Hospital between January 1990 and March 2011. RESULTS: Data from 52 CSS patients were analyzed. The respiratory tract was the most commonly involved organ (90.4%). Renal involvement was less frequent in antineutrophilic cytoplasmic antibody (ANCA)(-) patients than in ANCA(+) patients (p = 0.048). Clinical remission occurred in 95.3% of patients, but 16.3% of them relapsed. Patients who maintained remission for more than 6 months were relatively older (median, 51 years) at diagnosis (p = 0.004), had been diagnosed in earlier stages (p = 0.027), showed more frequent respiratory involvement (p = 0.024) and generalized symptoms (p = 0.039), and showed less frequent cutaneous involvement (p = 0.030) than those who did not achieve persistent (> 6 months) remission. Patients who achieved persistent remission also showed higher C-reactive protein (CRP) levels (p = 0.031) than those who did not. CONCLUSIONS: ANCA(-) CSS patients showed less frequent renal involvement. Characteristics of good responders were older age, diagnosis at earlier stages, less cutaneous involvement, more respiratory involvement, high CRP values, and more generalized symptoms.
Adolescent ; Adult ; Aged ; Antibodies, Antineutrophil Cytoplasmic/blood ; Churg-Strauss Syndrome/drug therapy/*epidemiology/immunology ; Female ; Glucocorticoids/therapeutic use ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Middle Aged ; Prednisolone/therapeutic use ; Republic of Korea/epidemiology ; Retrospective Studies ; Young Adult

OBJECTIVEAnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a disorder with poor prognosis. This study aimed to improve the diagnosis and treatment of ANCA associated vasculitis of children, to analyze the clinical features, pathological characteristics and the prognosis of children with ANCA-associated vasculitis.

METHODFifteen children with ANCA associated vasculitis who were hospitalized from 2003 to 2012 in our hospital were included. Their data of pre-diagnosis status, clinical manifestations, renal pathology, treatment and prognosis were reviewed retrospectively.

RESULTOf the 15 children, 11 were girls and 4 boys with a mean age of 10.7 years. Fourteen children were categorized as microscopic polyangitis. The time to diagnosis varied from 0.5 month to 40 months. Hematuria and proteinuria were revealed by urine analysis in all of them, only 6 children complained with gross hematuria or edema of oliguria. Decreased glomerular filtration rate was revealed in 13 children, 8 of whom had a creatinine clearance rate of less than 15 ml/(min·1.73 m(2)). Twelve children underwent renal biopsy, crescent formation was found in 11 children. Most of the crescents were cellular fibrous crescents or fibrous crescents. Six children were diagnosed as crescentic nephritis; the process of rapidly progressive nephritis was only observed in 2 children. Segmental glomerulosclerosis or global glomerulosclerosis were found in 10 children, 3 of them were diagnosed as sclerotic glomerulonephritis. Anemia and pulmonary injury were the most common extra renal manifestations. Other extra renal manifestations included rash, pain joint, gastrointestinal symptoms, abnormal findings of cardiac ultrasonography and headache. Eight children were treated with steroid combined with cyclophosphamide, 4 were treated with steroid and mycophenolate mofetil, 2 were treated with steroid, cyclophosphamide and mycophenolate mofetil, 3 children were treated with plasma exchange. Fourteen children were followed up for 0.5 month to 4 years. The renal function did not recover in children with creatinine clearance rate of less than 30 ml/(min·1.73 m(2)), who showed crescentic glomerulonephritis or sclerotic glomerulonephritis. The children who had creatinine clearance rate of more than 30 ml/(min·1.73 m(2))had better prognosis.

CONCLUSIONMore attention should be paid to ANCA-associated vasculitis among school age girls with anemia or pulmonary diseases. The renal damage was serious in children; however, the clinical manifestations were not obvious. Children with a creatinine clearance rate of less than 30 ml/(min·1.73 m(2)) had poor prognosis. Early accurate diagnosis is very important.

Adolescent ; Anemia ; etiology ; pathology ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; complications ; diagnosis ; pathology ; Antibodies, Antineutrophil Cytoplasmic ; blood ; immunology ; Biopsy ; Child ; Child, Preschool ; Creatinine ; blood ; Female ; Glomerulonephritis ; pathology ; Hematuria ; etiology ; pathology ; Humans ; Kidney ; pathology ; physiopathology ; Kidney Function Tests ; Male ; Nephritis ; diagnosis ; etiology ; pathology ; Prognosis ; Proteinuria ; etiology ; pathology ; Retrospective Studies

Aged ; Antibodies, Antineutrophil Cytoplasmic ; blood ; Comorbidity ; Glomerulonephritis ; etiology ; immunology ; physiopathology ; Humans ; Male ; Neutrophils ; pathology ; Thrombotic Microangiopathies ; diagnosis ; physiopathology

Drug-induced neutropenia (DIN), particularly that in which antibiotic-dependent antineutrophil antibodies have been detected, is a rare disorder. We report the case of a child with pneumococcal pneumonia, who experienced severe neutropenia during various antibiotic treatments. We detected 4 kinds (cefotaxim, augmentin, vancomycin, and tobramycin) of antibiotic-dependent antineutrophil antibodies by using the mixed passive hemagglutination assay (MPHA) technique with this child.
Anti-Bacterial Agents/*therapeutic use ; Antibodies, Antineutrophil Cytoplasmic/*blood/immunology ; Autoantibodies/blood/immunology ; Drug Therapy, Combination ; Humans ; Infant ; Male ; Neutropenia/chemically induced/*diagnosis ; Pneumonia, Pneumococcal/complications/*drug therapy ; Tomography, X-Ray Computed

Actins ; immunology ; Adolescent ; Adult ; Aged ; Antibodies, Antineutrophil Cytoplasmic ; blood ; Antibodies, Antinuclear ; blood ; Autoantibodies ; blood ; Child ; Child, Preschool ; Female ; Hepatitis, Autoimmune ; diagnosis ; immunology ; Humans ; Male ; Middle Aged ; Muscle, Smooth ; immunology

Propylthiouracil (PTU) is known to be a potential cause of antineutrophil cytoplasmic antibody (ANCA) positive small vessel vasculitis, resulting in glomerulonephritis and diffuse alveolar hemorrhage (DAH). Herein, we describe a 25-year-old pregnant woman who developed a perinulcear ANCA (p-ANCA) and myeloperoxidase ANCA (MPO-ANCA) positive DAH during PTU therapy. The patient improved after corticosteroid therapy and discontinuation of the PTU. Methimazole was prescribed in spite of the risk of recurrence of DAH because of the pregnancy. The patient is currently free from pulmonary problems. Our case shows that the alternative agent, methimazole, can be used to treat hyperthyroidism in a pregnant patient with PTU associated DAH.
Tomography, X-Ray Computed ; *Pulmonary Alveoli ; Propylthiouracil/*adverse effects/therapeutic use ; *Pregnancy Complications, Hematologic ; Pregnancy ; Hyperthyroidism/blood/complications/*drug therapy ; Humans ; Hemoptysis/*chemically induced/diagnosis/immunology ; Female ; Diagnosis, Differential ; Bronchoscopy ; Antithyroid Agents/*adverse effects/therapeutic use ; Antibodies, Antineutrophil Cytoplasmic/*blood ; Adult

OBJECTIVETo analyze the clinical features of patients with lupus nephritis positive for antineutrophil cytoplasmic antibodies (ANCA) and explore the clinical implications of ANCA detection.

METHODSTotally 261 patients with lupus nephritis were enrolled in this study, including 53 ANCA-positive and 208 ANCA-negative ones. The clinical data of the patients pertaining to the disease history, physical examination, laboratory examinations and pathological inspection were retrospectively analyzed.

RESULTSCompared with patients negative for ANCA, the ANCA-positive patients had significantly higher incidence of serositis (75.5%), acute renal failure (64.2%), myocarditis (30.2%), neuropsychiatric involvement (26.4%) and lung hemorrhage (7.5%)(P<0.05). Significant differences were also found between the two groups in SLE disease active index (SLE-DAI), number of the diagnostic criteria, erythrocyte sedimentation rate (ESR), anemia, anti-Sm antibodies, and serum complement C(3). Most patients positive for ANCA (67.9%) had type IV lupus nephritis with more crescent formation, renal tubular atrophy, hyaline thrombi, and higher mortality rate as well than the negative patients.

CONCLUSIONANCA detection may benefit the estimation of the disease severity and prognostic evaluation of lupus nephritis.

Adolescent ; Adult ; Antibodies, Antineutrophil Cytoplasmic ; blood ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Immunologic Factors ; blood ; Lupus Nephritis ; immunology ; pathology ; Male ; Prognosis ; Retrospective Studies

Behcet's disease (BD) is a multisystemic chronic inflammatory disease. It is characterized by recurrent oral and genital ulcers, uveitis, skin lesions and other manifestations, including neurologic, vascular, joint, and gastrointestinal ulcers of variable severity. Recurrent aphthous ulcer (RAU) represents a very common, but poorly understood, mucosal disorder. If a patient of RAU without any other typical symptoms of BD has gastrointestinal symptoms, it is difficult to distinguish this RAU from true BD with gastrointestinal involvement. Because pathognomonic clinical features and tools are absent, the differential diagnosis of these two diseases relies on the characteristic clinical features and the judgement of an experienced physician. Sixty-five out of a total 960 RAU patients and forty-four of 556 BD patients with gastrointestinal symptoms between January 1996 and December 2003 participated in this study. All were evaluated with esophagogastroduodenoscopy and colonoscopy. Clinical, endoscopic and histopathologic findings were analyzed and ELISA tests were conducted to detect serum levels of ASCA and pANCA. No significant difference was found between the two groups. Differential diagnosis between RAU with gastrointestinal symptoms and BD with gastrointestinal involvement requires further prospective, large-scale study.
Adolescent ; Adult ; Aged ; Antibodies, Antineutrophil Cytoplasmic/blood ; Antibodies, Fungal/blood ; Behcet Syndrome/*diagnosis/immunology/pathology ; Comparative Study ; Diagnosis, Differential ; Endoscopy ; Female ; Gastrointestinal Diseases/*diagnosis/immunology/pathology ; Humans ; Male ; Middle Aged ; Saccharomyces cerevisiae/immunology ; Serologic Tests ; Stomatitis, Aphthous/*diagnosis/immunology/pathology