PURPOSE: We investigated whether serum levels of insulin growth factor-1 (IGF-1) and insulin growth factor binding protein-3 (IGFBP-3) are valuable in predicting clinical outcomes or are correlated with other laboratory findings in children with Henoch-Schönlein purpura (HSP). METHODS: We examined 27 children who were consecutively admitted to our hospital with HSP between January 2011 and February 2012. Blood tests (C-reactive protein, white blood cell count, platelet count, erythrocyte sedimentation rate, albumin, immunoglobulin A, complement C3, antineutrophil cytoplasmic antibody, IGF-1, IGFBP-3) and urine tests were performed upon admission. IGF-1 and IGFBP-3 were resampled in the recovery phase. Controls included 473 children whose IGF-1 and IGFBP-3 were sampled for evaluating their growth, at the outpatient department of pediatric endocrinology in our hospital. IGF-1 and IGFBP-3 were compared between the HSP children and controls, and between the acute and recovery phases in HSP children. The ability of these values to predict clinical outcomes including renal involvement was analyzed using bivariate logistic regression analysis (BLRA). RESULTS: IGF-1 and IGFBP-3 were not different between the HSP children and controls (148.7±117.6 vs. 69.2±96.9, P=0.290: 3465.9±1290.9 vs. 3597.2±1,127.6, P=0.560, respectively). There was no significant difference in IGF-1 or IGFBP-3 between acute and recovery phases. Based on the BLRA, no variable, including IGF-1 and IGFBP-3, could predict clinical outcomes including the presence of nephritis. CONCLUSION: We concluded that IGF-1 and IGFBP-3 do not predict clinical outcomes of HSP, including renal involvement, in this study.
Antibodies, Antineutrophil Cytoplasmic ; Blood Sedimentation ; Child* ; Complement C3 ; Endocrinology ; Hematologic Tests ; Humans ; Immunoglobulin A ; Insulin* ; Insulin-Like Growth Factor Binding Protein 3 ; Insulin-Like Growth Factor I ; Leukocyte Count ; Logistic Models ; Nephritis ; Outpatients ; Platelet Count ; Purpura*

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

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*

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

The systemic vasculitides are a group of diverse diseases characterized by blood vessel inflammation. The existing classification criteria are intended to create homogeneous patient groups for research and not to diagnose individual patients. However, they have been misused as diagnostic criteria, in both practice and research. The existing classification systems for vasculitis are limited by the overlapping features of disease entities and unrecognized pathogenic mechanisms. This review discusses the benefits and limitations of the widely used American College of Rheumatology criteria and Chapel Hill Consensus Conference nomenclature, updated in 2012. Improved diagnostics, including antineutrophil cytoplasmic antibody (ANCA) testing and imaging, argue for updating the established classification criteria. International efforts are underway to build a more effective classification and diagnostic criteria that reflect a better understanding of the pathophysiology of vasculitis and recent discoveries of genetics and biomarkers.
Antibodies, Antineutrophil Cytoplasmic ; Biomarkers ; Blood Vessels ; Classification* ; Consensus ; Genetics ; Humans ; Inflammation ; Rheumatology ; Systemic Vasculitis ; Vasculitis*

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

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

Thrombotic microangiopathy (TMA) is a microvascular thrombotic lesion caused by endothelial injury and subsequent formation of platelet rich thrombus. TMA is first described as a classical pathologic feature of HUS/TTP. Renal biopsy finding of TMA represents kidney involvement of HUS/TTP as well as other diseases such as malignant hypertension, drug toxicity, eclampsia, pre-eclampsia, and several systemic infections. Autoimmune diseases and transplant kidney sometime also have TMA. It is needed to consider a complete autoimmune work-up of patients presenting with TMA including tests for ANA, ANCA, and ADAMTS13 inhibitory antibodies, because there are several reports of association with TMA in patients of SLE, anti-phospholipid syndrome, and ANCA-associated vasculitis.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies ; Antibodies, Antineutrophil Cytoplasmic ; Antiphospholipid Syndrome ; Autoimmune Diseases ; Biopsy ; Blood Platelets ; Drug Toxicity ; Eclampsia ; Female ; Humans ; Hypertension, Malignant ; Kidney ; Pre-Eclampsia ; Pregnancy ; Thrombosis ; Thrombotic Microangiopathies ; Transplants

Henoch-Schonlein purpura (HSP) is common in childhood and often self-limiting. There have been limited studies on elderly-onset HSP nephritis (HSPN). A 76-yr-old man was transferred to our hospital with a 1-month history of oliguria, abdominal pain, edema and palpable purpura in the legs. Three months ago, he was admitted to another hospital with jaundice, and consequently diagnosed with early common bile duct cancer. The patient underwent a Whipple's operation. Antibiotics were administrated because of leakage in the suture from the surgery. However, he showed progressive renal failure with edema and purpura in the legs. Laboratory investigations showed serum creatinine 6.4 mg/dL, 24-hr urine protein 8,141 mg/day, myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA) 1:40 and C3 below 64.89 mg/dL. Renal biopsy showed crescentic glomerulonephritis, as well as mesangial and extracapillary Ig A deposition. We started steroid therapy and hemodialysis, but he progressed to end-stage renal failure and he has been under maintenance hemodialysis. We describe elderly onset HSPN with MPO-ANCA can be crescentic glomerulonephritis rapidly progressed to end stage renal failure.
Aged ; Antibodies, Antineutrophil Cytoplasmic/*analysis ; Common Bile Duct Neoplasms/complications/surgery ; Complement C3/analysis ; Creatinine/blood ; Edema/drug therapy ; Enzyme-Linked Immunosorbent Assay ; Glomerulonephritis/pathology ; Humans ; Male ; Purpura, Schoenlein-Henoch/*diagnosis/drug therapy ; Renal Dialysis ; Renal Insufficiency/etiology/pathology ; Steroids/therapeutic use

OBJECTIVETo study the value of anti-neutrophil cytoplasmic antibody (ANCA) in diagnosis of Kawasaki disease (KD).

METHODSSerum ANCA was detected in 30 children with typical Kawasaki disease (TKD) and in 16 with incomplete Kawasaki disease (IKD) in the acute and the recovery phases respectively. Twenty-five healthy children were randomly selected as a control group. An ultrasonic cardiography (UCG) was performed on children with KD in the acute phase.

RESULTSThe mean positive rate of serum ANCA in the acute phase in KD children was 65%, with 69% in IKD children and 63% in TKD children, which were obviously higher than that in the control group (P<0.01). The positive rate of serum ANCA in the recovery phase in KD children was significantly lower than that in the acute phase (33% vs 65%, P<0.05). The positive rate of serum ANCA in the acute phase in children with KD was significantly higher than that detected by UCG (P<0.01). The incidence rate of coronary artery lesions in children with positive ANCA was obviously higher than that in children with negative ANCA (43% vs 13%; P<0.05).

CONCLUSIONSSerum ANCA may be used as a reference index for early diagnosis of KD and secondary coronary artery lesions in children.

Antibodies, Antineutrophil Cytoplasmic ; blood ; Child ; Child, Preschool ; Echocardiography ; Female ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; blood ; diagnosis