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 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

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

Recent reports have indicated that a significant number of immune complex glomerulonephritis (GN) cases are associated with antineutrophilic cytoplasmic antibody (ANCA). However, most of the reported cases were associated with underlying primary glomerular diseases. When primary glomerular diseases were not found, immune deposits tended to be non-specific and the level of ANCA is usually borderline. We report here upon a case of life-threatening pulmonary-renal syndrome manifested simultaneously with immune complex GN and myeloperoxidase (MPO)-ANCA seropositivity. A 29- year-old man was admitted with pulmonary hemorrhage and rapidly progressing renal dysfunction. On admission, ANCA revealed perinuclear staining with a titer of 1:160. The MPO-ANCA level was 59 IU by ELISA. Other serologic markers including ANA, anti-DS-DNA and anti-GBM Ab were negative. Renal biopsy showed cellular crescents in eight of 18 glomeruli. Immunofluorescence staining showed strong granular deposits of C3, C1q, IgG and IgM in the capillary loop and the mesangium. Electron microscopy showed multifocal electron dense deposits scattered in the mesangium, paramesangium, and the subendothelial and subepithelial areas. The patient initially responded to steroid and cyclophosphamide. MPO-ANCA decreased to less than 10 IU. Twenty three days after hospital discharge, the patient was re-admitted urgently with fever, generalized papulonodular skin lesions, and a recurrence of massive pulmonary hemorrhage and renal dysfunction. He died from uncontrolled pulmonary hemorrhage and respiratory insufficiency. P-ANCA titer and MPO-ANCA level at the second admission were 1:320 and 82 U/ml respectively. Interestingly, relapse was shown to be triggered by varicella zoster infection.
Adult ; Antibodies, Antineutrophil Cytoplasmic/*blood ; Antigen-Antibody Complex/*metabolism ; Glomerulonephritis/*etiology ; Hemorrhage/complications ; Human ; Lung Diseases/blood/*complications ; Male ; Peroxidase/*blood

Antibodies, Antineutrophil Cytoplasmic ; blood ; Child ; Female ; Hematuria ; etiology ; Humans ; Kidney ; pathology ; physiopathology ; Kidney Function Tests ; Proteinuria ; etiology ; Renal Insufficiency ; etiology ; Vasculitis ; blood ; complications ; pathology

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

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

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

Anti neutrophil cytoplasmic antibody (ANCA)-positive vasculitis and crescentic glomerulonephritis has been rarely reported in patients suffering from Graves' disease and treated with Propylthiouracil. We experienced a case of ANCA-positive crescentic glomerulonephritis presenting good prognosis after discontinuing Propylthiouracil. A 40-year-old female visited due to the proteinuria and hematuria in urinalysis. She had been medicated Propylthiouracil for 3 years. Blood pressure was 100/60 mmHg. BUN and serum creatinine were 24.7 mg/dL, and 1.9 mg/dL, respectively. Urinalysis revealed protein 1481 mg/day, many RBC's/HPF (dysmorphic 80%), Serological ANCA was positive, anti-myeloperoxidase (MPO) antibody 1,922 AAU/ mL (normal <150 AAU/mL). The histologic finding showed crescentic glomerulonephritis on light microscopy, but no immuno deposit on immunofluorescence and light microscopy. So we diagnosed ANCA positive pauci-immune glomerulonephritis. Propylthiouracil was discontinued and steroid, cyclophosphamide was medicated within about 1 month, but stopped due to cytopenia. Patient's creatinine level was maintained 1.3 mg/dL and showed stable progress for about over 18 months. We report this case that showed good prognosis after discontinuation of Propylthiouracil.
Adult ; Antibodies, Antineutrophil Cytoplasmic ; Blood Pressure ; Creatinine ; Cyclophosphamide ; Female ; Fluorescent Antibody Technique ; Glomerulonephritis ; Graves Disease ; Hematuria ; Humans ; Light ; Microscopy ; Prognosis ; Propylthiouracil ; Proteinuria ; Stress, Psychological ; Urinalysis ; Vasculitis

BACKGROUND: The vasculitis are a group of diseases and disorders sharing the central feature of inflammation of the blood vessel wall with attendant tissue ischemia. The purpose of this study was to determine the types and frequency of neurological involvement in patients with vasculitis. METHODS: We reviewed the medical records of 131 patients with vasculitis who were examined at Severance Hospital. RESULTS: The nervous system was involved in 47 out of 131 cases. Patients with systemic necrotizing vasculitis showed the highest frequency of neurological involvement (78.3%) of which peripheral involvement was the most common. Patients with Wegener's granulomatosis showed 36.4% of neu-rological involvement of which all cases were cranial nerve palsy. Neurological involvement was found in 18.7% of cases with Takayasu's arteritis and was limited to the central nervous system. Neurological symptoms were initial mani-festations in 56.5% of cases with systemic necrotizing vasculitis, 9% with Wegener's granulomatosis, and 2.7% with Takayasu's arteritis. Among various laboratory values, positive rate of p-ANCA was significantly higher in Wegener's granulomatosis cases with neurological involvement than cases without involvement. CONCLUSIONS: We found that the frequency and distribution of neurological involvement vary with the underlying disorder. Neurological manifestations may provide an important clue for the diagnosis of systemic necrotizing vasculitis because neurological involvements frequently occur as an initial manifestation of it. (J Korean Neurol Assoc 19(4):370~375, 2001)
Antibodies, Antineutrophil Cytoplasmic ; Blood Vessels ; Central Nervous System ; Cranial Nerve Diseases ; Diagnosis ; Humans ; Inflammation ; Ischemia ; Medical Records ; Nervous System ; Neurologic Manifestations ; Peripheral Nerves ; Takayasu Arteritis ; Vasculitis ; Wegener Granulomatosis