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]

Synonyms and Keywords: : Place different synonyms or keywords you think readers may search to arrive to this page here 

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 

References


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