Today I saw a news article: “Qigong master” Wang Lin died of ANCA-associated vasculitis and autoimmune peripheral neuritis, which led to multi-organ failure. Many of my friends asked me, “What is ANCA-associated vasculitis in the end a disease? Even the master can’t manage it. As a rheumatologist, I feel it is important to educate you about this “common disease” in rheumatology. The first thing we need to know about ANCA is that it is called Anti-Neutrophil Cytoplasmic Antibodies, and there are many kinds of target antigens for ANCA, the most common ones are Proteinase 3 and Myeloperoxidase, which are also known as C-ANCA. ANCA is an important indicator for diagnosis, monitoring of disease activity and prevention of relapse. Granulomatous polyangiitis, eosinophilic granulomatous polyangiitis, and microscopic polyangiitis (formerly called Wegener’s granulomatosis, allergic granulomatous vasculitis, and microscopic polyangiitis, respectively) are three diseases with similar clinical presentation, treatment, and prognosis, and most patients are ANCA-positive, hence the name ANCA-associated vasculitis. ANCA-associated vasculitis is relatively common in whites and is not uncommon in our country. The average age of diagnosis is 55 years, but it is not uncommon in older adults over 80 years of age. Because of the complex clinical presentation, it is easily misdiagnosed. A statistic from Peking University People’s Hospital shows that only 3% of patients are initially diagnosed in the department of rheumatology. ANCA-associated vasculitis is an autoimmune disease with multisystem involvement, and multiple systems throughout the body can be involved. Let me tell you a little bit about it from head to toe. 1. Eyes: sclerositis, orbital swelling, nasolacrimal duct obstruction; 2. Ears: hearing loss; 3. Nose: bloody runny nose, sinusitis, oronasal ulcers, allergic rhinitis, polyps; 4. Tracheobronchial: subglottic or bronchial stenosis; 5. Lung: dysphagia, cough, hemoptysis, asthma, interstitial lung lesions and pulmonary infiltrative foci; 6. Heart: pericarditis, valvulitis, infarction, cardiomyopathy; 7. Gastrointestinal: eosinophilic acid granulocytic gastroenteritis; 8, kidney: hematuria, proteinuria, renal insufficiency, and in some patients, acute renal failure, which is a more serious condition in ANCA-associated arthritis; 9, arthritis/arthralgia; 10, central nervous system: cerebral neuropathy, occupancy, meningitis, cerebral ischemia; 11, peripheral nerves: polyneuritis mononeuritis and disabling sensory polyneuropathy; 12, hematologic system: eosinophilia; 13, skin: purpura, ulcers, limb infarcts and lamellar hemorrhage; 14, systemic lesions: fever, malaise, weight loss. From the above clinical manifestations, the patient is likely to have lesions from head to toe and from skin to internal organs. A confirmed diagnosis of vasculitis requires aggressive hormone and immunosuppressive therapy, and some more severe patients may require high-dose hormone or gammaglobulin shock therapy. Despite aggressive treatment, the mortality and disability rates of ANCA-associated vasculitis are high. The 5-year survival rate for patients is only 76%. Early detection, early diagnosis, and aggressive treatment are key.