ANCA associated vasculitis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]

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Overview

Brief overview describing the disease entities, their similarities and differences here.

	Eosinophilic granulomatosis with polyangiitis	Granulomatosis with polyangiitis	Microscopic polyangiitis
Classification	According to the American College of Rheumatology 4 out of 6 criteria need to be established: ·         Asthma ·         Eosinophilia >10 ·         Neuropathy (mononeuropathy or polyneuropathy) ·         Pulmonary infiltration, non-fixed ·         Paranasal sinus abnormality ·         Biopsy with extravascular eosinophilic infiltration Lanham’s criteria, all of the following need to be present: ·         Asthma ·         Eosinophilia peak of >1.5x109cell/L ·         Systemic vasculitis, two or greater extra pulmonary sites	According to the American College of Rheumatology 2 out of 4 criteria need to be established: ·         Abnormal urinary sediment ( red cell casts or > 5 red blood cells per hpf) ·         Abnormal chest radiography ( nodules, cavities, or fixed infiltrates) ·         Nasal discharge or oral ulcers ·         Biopsy with granulomatous inflammation According to the European League Against Rheumatism, Pediatric Rheumatology European Society and Pediatric Rheumatology International Trials Organization three out of the six criteria are established: ·         Histopathology shows granuloma ·         Upper respiratory tract involvement ·         Stenosis of larynx, trachea and bronchioles ·         Pulmonary involvement ·         Anti-neutrophil cytoplasmic antibodies ·         glomerulonephritis
Epidemiology	Prevalence: 2 to 3 per million persons; higher prevalence’s seen in Europeans. Incidence: 0.5 to 6.8 per million persons Age: Mean age 48 years; incidence occurs in females between 1st decade to 7th decade. Males are similarly as females. Gender: no sex predominance is seen Race: Prevalence of the disease is seen in both Asiatic and European populations; displaying equal distribution.	Prevalence: Europe: 23.7 to 160 per million persons. Prevalence in the United States: 3 per 100,000 persons. Incidence: Europe: 3 to 14.4 per million persons Children: 0.03 to 3.2 per 100,000 persons Age: Mean age 58 years; incidence occurs in females during 7th decade and males in the 8th decade. Commonly seen in middle aged and elderly patients. In children the ages are 4 to 17 however, the disease is rarely seen. Gender: Males are more commonly affected; the overall ratio is 1.2 to 1. However, the ratio is dependent upon geographical location. In children females are commonly seen with the disease. Race: Affects Caucasian race, other races may be affected however the rates are lower.	Prevalence: 1 to 3 per 100,000 persons. Southern Sweden: 94 per million persons Incidence: Spain: 11.6 per million persons United Kingdom: 5.9 per million persons United States: 3.6 per millions persons Norway: 2.7 per million persons *during the last 2 decades the rate of incidences have increased, which is attributed to the availability of ANCA testing. Age: seen during the 5th and 6th decade of life Gender: Males are more commonly affected than females; overall ratio is 1.8 to 1. Race: Typically seen in patients of Asiatic and southern European descents.
Signs and symptoms	Prodromal phase: Arthralgia, myalgia, pyrexia, weight loss, asthma, nasal polys, allergic rhinitis, sinusitis, otitis media, sensorineural hearing loss, asthenia Eosinophilic phase: Peripheral eosinophilia, pulmonary infiltrates, ground glass opacities, thickening of bronchial wall, endomyocardial infiltrates, arrhythmia, pericarditis, abdominal pain Vascular phase: Pyrexia, weight loss, fatigue, multiplex mononeuritis, cerebral infarcts, rapid progressive glomerulonephritis, pauci-immune focal and segmental necrotizing glomerulonephritis, palpable purpura and nodules, maculopapular erythema, Livedo reticularis, petechiae, ecchymoses	Constitutional symptoms: Malaise, pyrexia, weight loss, arthralgia, cough, dyspnea, purpura, and abnormal urinary sediment Renal involvement: Glomerulonephritis, hematuria, elevated serum creatinine, edema, hypertension, red cell casts	Commonly seen: Renal inflammation (glomerulonephritis), weight loss, skin lesions, nerve damage, pyrexia. Renal involvement: Hematuria, proteinuria, red cell casts
Complications	Pericarditis, myocarditis, myocardial infarction, heart failure, cerebral hemorrhage, gastrointestinal bleeding, status asthmaticus	Vision loss, subglottic manifestations, auditory loss, renal failure, infections due to prolong immunsuppressants	Abdominal sepsis, meningoencephalitis, alveolar hemorrhage, osteoarticular disease, end stage renal failure.
Physical examination	Hypertension, palpable purpura, nodules, nasal polyps, allergic rhinitits, paranasal sinsusitis, asthma, pneumonitis, hemoptysis, myocarditis, uremia, gastroenteritis, bowel ischemia/ perforation, numbness or tingling of extremities, peripheral neuropathy	Sinusitis, scleritis, keratitis, uveitis, conjunctivitis, saddle nose deformity, atelectasis, pleural effusion, dyspnea, hemoptysis, pericardial rub, mononeurtitis multiplex, cranial nerve paralysis, palpable purpura, subcutaneous nodules, ulcerations	Pyrexia, leukocytoclastic angiitis, palpable purpura, livedo reticularis, necrosis, necrotizing nodules, rales at the base of lungs, sinusitis, hypertension, myocardial infarction, pericarditis, gastrointestinal bleeding, ischemia/ perforation of bowel, pancreatitis, retinal hemorrhage, scleritis, uveitis, uremia, mononeuritis multiplex, orchitis
Prognosis	If left untreated, the disease is fatal. Patients receiving no treatment have an estimated survival rate of 20 to 30%. The prognosis is heavily dependent upon starting therapy.	If left untreated, the disease may progress to morbidity and mortality as soon as 4 weeks, with an average estimated mortality of 5 months. In the past the survival for 1 year was 18%, today the survival rate is estimated at 95% for 1 year, 83% for 5 years and 65% for 10 years.	If left untreated, the disease is fatal. Patients receiving no treatment have an estimated survival rate of 10% in 2 years. The mortality rate today is 12% for 4 years and 44% in 10 years due to the advent of glucocorticoids and cyclophosphamide.
Gross pathology	Nodular swelling along small arteries (heart, liver, and renal) Infarcts, hemorrhage and scarring of affected organs Pulmonary artery occlusion Patchy consolidation (lower portion of lung) Fibrosis, ventricular hypertrophy of both ventricles, patchy myocardial scars, and endocardial fibrosis	Ulceration necrosis of bone and cartilage vascular necrosis	Hemorrhagic necrotizing alveolar capillaries Fibrinous necrosis of lung Intra-alveolar hemosiderosis
Microscopic pathology	Eosinophilic infiltrates with necrosis Giant cell vasculitis with eosinophils Interstitial and perivascular necrotizing granulomas Eosinophilic lymphadenopathy	Focal and segmental necrotizing glomerulonephritis presence of non-caseating granuloma necrotizing vasculitis at times varied multinucleated giant cells	Focal segmental necrotizing glomerulonephritis Crescent glomeruli Minimal deposition of immunoglobulins Compliment in glomeruli and renal vasculature
Serological findings	Perinuclear ANCA (pANCA) 30-40% positive Myeloperoxidase antigen 40% sensitivity Proteinase 3 antigen <5% sensitivity	Cytoplasmic ANCA (cANCA) 90% positive Myeloperoxidase antigen 10% sensitivity Proteinase 3 antigen 70-80% sensitivity	Perinuclear ANCA (pANCA) 60-80% positive Myeloperoxidase antigen 30% sensitivity Proteinase 3 antigen 60% sensitivity
Laboratory findings	Anti-neutrophil cytoplasmic antibodies, hypereosinophilia, elevated immunoglobulin E titers (IgE)	Anti-neutrophil cytoplasmic antibodies, elevated blood urea nitrogen, elevated serum creatinine, erythrocyte sedimentation rate, C reactive protein, proteinuria, microscopic hematuria, red blood casts	Anti-neutrophil cytoplasmic antibodies, elevated blood urea nitrogen, elevated serum creatinine, elevated erythrocyte sedimentation rate, proteinuria, hematuria, red cell casts, leukocytosis
Biopsy	Renal biopsy is gold standard in establishing the disease.	Renal biopsy is gold standard in establishing the disease. Under electron microscopy: subendothelial edema, microthrombosis, and degranulation of neutrophils. Light microscopy: necrotizing and crescentic glomerulonephritis	Biopsy of involved skin, lung, renal and nerve can be made to establish the disease. Skin biopsy: immunoglobulins and complement components Renal biopsy: crescent formation with focal necrosis Lung biopsy: Alveolar capillaritis Nerve biopsy: Vascular necrosis of small and medium sized vessels
Treatment	Induction: Mild form Glucocorticoid Methotrexate Azathioprine Hydroxyurea Mycophenolate Severe form  Glucocorticoid + cyclophosphamide Second-line therapy: IV immunoglobulins + glucocorticoids + immunosuppressant in patients with flare-ups due to certain medication or during pregnancy. Maintenance: Azathioprine Methotrexate	Induction: Glucocorticoid Cyclophosphamide Rituximab Maintenance: Azathioprine Methotrexate Rituximab	Induction: Glucocorticoid + cyclophosphamide Severe form  Glucocorticoid + immunosuppressant + plasmapheresis Maintenance: Myclophenolate mofetil Methotrexate