Primary antineutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV) constitute a group of small vessel vasculitides that includes Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome. Recently, many in vitro and in vivo studies have highlighted the role of ANCA as the main pathophysiological factor in the development of AAV. Two remarkable studies on ANCA pathogenesis were recently reported. One study examined anti-lysosomal membrane protein-2, which supports the 'shared epitope' theory. The other examined the neutrophil extracellular trap that is released by neutrophils primed by ANCA. Each disease of AAV shows a broad spectrum of the clinical features and severities, which makes it difficult to diagnose and treat them. Considerable effort has been made in the past decades to improve the treatment outcomes, reduce the incidence of relapse and avoid drug toxicity. This review describes the current understanding of AAV along with a few Korean reports.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Churg-Strauss Syndrome ; Drug Toxicity ; Glycosaminoglycans ; Incidence ; Membranes ; Microscopic Polyangiitis ; Neutrophils ; Recurrence ; Vasculitis ; Wegener Granulomatosis

PURPOSE: Delta neutrophil index (DNI) represents the immature granulocytes count associated with neutrophil-consumption. We investigated whether DNI might be associated with Birmingham vasculitis activity score (BVAS) at diagnosis and could predict relapse during the follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). MATERIALS AND METHODS: We reviewed the medical records of 97 patients having DNI results. Twenty patients had granulomatosis with polyangiitis (GPA), 58 had microscopic polyangiitis (MPA), and 19 had eosinophilic GPA (EGPA). We collected clinical and laboratory data including BVAS, five factor score (FFS), and DNI. The correlation coefficient and cumulative relapse free survival rate were obtained. The optimal cut-off of DNI was extrapolated by calculating the area under the receiver operator characteristic curve. RESULTS: DNI was significantly related to cross-sectional BVAS. Furthermore, among continuous variables, only DNI could reflect BVAS of GPA and MPA, but not EGPA. Severe AAV was defined as BVAS ≥20 (the highest quartile). At diagnosis, patients having DNI ≥0.65% had a significantly higher risk of severe GPA and MPA than those having not (relative risk 4.255) at diagnosis. During the follow-up, DNI ≥0.65% could predict the higher relapse rate. CONCLUSION: DNI could reflect BVAS at diagnosis and furthermore, DNI ≥0.65% could not only identify severe AAV at diagnosis, but also predict relapse during the follow-up in patients with GPA and MPA.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* ; Cytoplasm ; Diagnosis ; Eosinophils ; Follow-Up Studies ; Granulocytes ; Granulomatosis with Polyangiitis ; Humans ; Medical Records ; Microscopic Polyangiitis ; Neutrophils* ; Recurrence* ; Survival Rate ; Vasculitis*

OBJECTIVE: To investigate the significance of a set of seven disease activities and extension measurements and their correlations between one and another for anti-neutrophil cytoplasmic autoantibody associated vasculitis (AAV). METHODS: A total of 121 patients from Peking University International Hospital and Fouth Medical Center of PLA General Hospital with confirmed diagnoses of AAV clinically were enrolled in the study, including 15 cases of eosinophilic granulomatous with polyangiitis (EGPA), 59 cases of granulomatous with polyangiitis (GPA) and 47 cases of microscopic polyangiitis (MPA). A hundred and twenty-one AAV patients were divided into death group and survival group according to their survival conditions. A set of seven disease assessment scales including Birmingham vasculitis activity score (BVAS)-1994, BVAS-2003, as well as BVAS/GPA, vasculitis damage index (VDI), disease extent index (DEI), five factor score (FFS)-1996, and FFS-2009 were measured and scored one by one, and their relationships which were represented by Spearman correlation coefficient were compared between one and another. RESULTS: BVAS-1994, BVAS-2003, as well as BVAS/GPA, VDI, DEI, and FFS, all of those seven evaluation indexes of the AAV patients in the death group were significantly higher than those in the survival group (P<0.05). Except for BVAS/GPA, all those above indicators in the patients with EGPA were lower than those in the patients with GPA and those in the patients with MPA, and those in all of the AAV patients as a whole group. There were high correlations among BVAS-2003, BVAS-1994 and BVAS/GPA (r values were 0.9 and 0.7, respectively); BVAS-1994 was fairly correlated with BVAS/GPA (r=0.69); FFS-1996 and FFS-2009 were highly correlated (r=0.73) with each other; BVAS-1994, BVAS-2003 and BVAS/GPA were fairly correlated with DEI (with r values of 0.62, 0.65, and 0.62, respectively); VDI was also fairly correlated with BVAS-1994 and with BVAS-2003 (r values were 0.49 and 0.52, respectively). CONCLUSION All of those seven AAV assessment indicators above can be used as indicators of disease activity and prognosis in AAV patients, most of which were relevant within one and another. There were high correlations among BVAS-2003, BVAS-1994 and BVAS/GPA, and besides, there were also high correlations between FFS-1996 and FFS-2009.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis* ; Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies ; Biomarkers/analysis* ; Humans ; Microscopic Polyangiitis ; Neutrophils

PURPOSE: We investigated whether C-reactive protein (CRP) to serum albumin ratio (CAR) could be an independent predictor of all-cause mortality in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). MATERIALS AND METHODS: We retrospectively reviewed the medical records of 170 patients with AAV. We collected clinical and laboratory data. We also examined AAV-related and traditional risk factors of all-cause mortality. To assess the hazard ratios of variables, we performed univariable and multivariable Cox hazard model analyses. RESULTS: The mean age was 55.0 years and 53 patients (31.2%) were male among 170 patients with AAV (88 microscopic polyangiitis, 43 granulomatosis with polyangiitis, and 39 eosinophilic granulomatosis with polyangiitis). ANCA was detected in 129 patients (75.9%). The initial mean CRP and serum albumin were 41.1 (mg/L) and 3.6 (g/dL), and the mean CAR at diagnosis was 14.8. The most common risk factor of mortality was hypertension (42.4%), followed by chronic kidney disease ≥stage 3 (25.9%). Fourteen patients (8.2%) died during the mean follow-up of 56.7 months. In both multivariable Cox hazard model analyses, CAR at diagnosis was identified as an independent predictor of all-cause of mortality comparable to diabetes mellitus (DM). Moreover, patients with CAR ≥10.35 and having DM exhibited a higher frequency of all-cause mortality than those without. CONCLUSION: CAR at diagnosis can be an independent predictor of all-cause mortality, comparable to DM, the conventional risk factor of mortality.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* ; Antibodies, Antineutrophil Cytoplasmic ; C-Reactive Protein* ; Diabetes Mellitus ; Diagnosis ; Eosinophils ; Follow-Up Studies ; Granulomatosis with Polyangiitis ; Humans ; Hypertension ; Male ; Medical Records ; Microscopic Polyangiitis ; Mortality* ; Proportional Hazards Models ; Renal Insufficiency, Chronic ; Retrospective Studies ; Risk Factors ; Serum Albumin* ; Vasculitis

Background: ANCA-associated vasculitis and its subtypes have been associated with pulmonary manifestations, with bronchiectasis being a unique clinical presentation. Case Summary We report the case of a 26-year-old Filipino male who presented with progressive dyspnea, neuropathic pain, and purpuric rash. Diagnostic evaluation revealed upper lobe bronchiectasis and lower lobe pneumonia, as well as hematuria and proteinuria. ANCA-associated vasculitis (AAV) and tuberculosis were considered. There was improvement of dyspnea, cough and rashes with antibiotics, glucocorticoids (GC), and anti-TB coverage. However, neuropathic pain progressed to the upper and lower extremities with development of weakness. Anti-myeloperoxidase (MPO) Anti-Neutrophil Cytoplasmic Antibody (ANCA) was positive, Electromyography-Nerve Conduction Velocity (EMG-NCV) revealed diffuse sensorimotor axonal polyradiculopathy of both upper and lower extremities. Cyclophosphamide was then given. The patient gradually regained his motor strength while sensory deficits persisted. He was referred to rehabilitation medicine for physical therapy and eventually discharged. This case highlights vasculitis as an associated extrapulmonary manifestation of bronchiectasis, and the possible role of bronchiectasis in the immune-mediated pathogenesis of ANCA- associated vasculitides.
Bronchiectasis ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis is a primary systemic vasculitis that affects the small vessels, and ANCA is involved as the common pathogenesis. Environmental factors such as infectious agents have been considered to play a role in triggering the autoimmunity. We report here on a case of ANCA-associated vasculitis that developed after scrub typhus. A 64-year-old male was admitted because of fever, chills, pain, weakness and hypoesthesia of his calves. He was diagnosed as having scrub typhus based on the findings of an eschar and the positive serum anti-orientia antibody. The fever continued despite the antibiotic treatment. Neurologic symptoms such as numbness, hypoesthesia and weakness began to develop in the hands, feet and calves with a persisting fever. The nerve conduction velocity study revealed mononeuritis multiplex of the superficial peroneal nerve and the median nerve. Microscopic hematuria then additionally developed, and the serology showed a positive myeloperoxidase (MPO) test. A nerve biopsy was conducted on the left superficial peroneal nerve and the result showed non-infectious systemic vasculitis of the medium-small arteries. He was diagnosed as having microscopic polyangiitis along with ANCA associated vasculitis. The fever resolved and the neurologic symptoms began to improve after steroid pulse treatment (methylprednisolone 1 g/day). The neuropathy gradually improved after discharge. We presume that the ANCA-associated vasculitis was triggered by scrub typhus.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Arteries ; Autoimmunity ; Biopsy ; Chills ; Cytoplasm ; Fever ; Foot ; Hand ; Hematuria ; Humans ; Hypesthesia ; Male ; Median Nerve ; Microscopic Polyangiitis ; Middle Aged ; Mononeuropathies ; Neural Conduction ; Neurologic Manifestations ; Peroneal Nerve ; Peroxidase ; Scrub Typhus ; Systemic Vasculitis ; Vasculitis

Microscopic polyangiitis is a systemic small-vessel vasculitis primarily associated with necrotizing glomerulonephritis and pulmonary capillaritis. This disease is determined by clinical manifestation, ANCA, and involved organ or renal biopsy. We describe a 49-year-old female presenting with chronic postnasal drip, progressive dyspnea and abrupt hemoptysis. Her serum p-ANCA was postitive, but c- ANCA was negative. Her chest X-ray and chest CT scan showed pulmonary hemorrhage, and the renal biopsy specimen revealed crescentic glomerulonephritis. She was diagnosed as having an ANCA-associated vasculitis, and more specifically, a microscopic polyangiitis accompanied with paranasal sinusitis. She was treated with intravenous methylprednisolone pulse therapy, followed by prednisolone and cyclophosphamide per oral tablets. She showed rapid progression of renal failure and died from sepsis after 2 months of treatment.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Cyclophosphamide ; Dyspnea ; Female ; Glomerulonephritis ; Hemoptysis ; Hemorrhage* ; Humans ; Methylprednisolone ; Microscopic Polyangiitis* ; Middle Aged ; Prednisolone ; Renal Insufficiency ; Sepsis ; Sinusitis* ; Tablets ; Thorax ; Tomography, X-Ray Computed ; Vasculitis

Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis associated with antineutrophil cytoplasmic autoantibodies. Interstitial lung disease is a less recognized manifestation of MPA and has a poor prognosis. A 61-year-old man presented with persistent cough, sputum and dyspnea. Laboratory examination revealed microscopic hematuria and renal insufficiency. Perinuclear anti-neutrophil cytoplasmic autoantibodies were positive according to serological testing. Computed tomography scans showed bibasilar reticulation and honeycombing in a peripheral distribution. Therefore, renal biopsy was performed, and MPA was diagnosed. After treating with corticosteroids and immunosuppressive agents, the patient had a complete renal response but progressive interstitial lung disease. We report a case of MPA presenting with interstitial lung disease in which the patient experienced different responses in each organ.
Adrenal Cortex Hormones ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Autoantibodies ; Autoimmune Diseases ; Biopsy ; Cough ; Cytoplasm ; Dyspnea ; Hematuria ; Humans ; Immunosuppressive Agents ; Lung Diseases, Interstitial* ; Microscopic Polyangiitis* ; Middle Aged ; Prognosis ; Renal Insufficiency ; Serologic Tests ; Sputum ; Systemic Vasculitis

No abstract available.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Hyperthyroidism ; Propylthiouracil

No abstract available.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Hyperthyroidism ; Propylthiouracil