Pulmonary vasculitis

Introduction to pulmonary vasculitis Vasculitis is a group of diseases in which inflammatory changes in the blood vessel wall are the main pathological manifestations. Vasculitis can cause vascular destruction, so it is sometimes called necrotic vasculitis. Vasculitis involves a wide range of diseases, either primary vasculitis or concomitant or secondary to other diseases; the invading blood vessels can be mainly arteries, but can also involve movements, veins and capillaries. Small blood vessels can be the main invasive object, or it can be a disease mainly caused by larger blood vessels. Pulmonary vasculitis, as the name implies, refers to vasculitis in which pulmonary blood vessels are invaded. Some pulmonary vasculitis is relatively rare, and diagnosis is difficult, which should cause clinical Pay enough attention. basic knowledge The proportion of illness: 0.003% Susceptible people: more common in the elderly over 60 years old Mode of infection: non-infectious Complications: keratitis

Cause of pulmonary vasculitis

(1) Causes of the disease

The cause is not clear.

(two) pathogenesis

The pathological feature of pulmonary vasculitis is the inflammatory reaction of the vessel wall, which often runs through the whole layer of the vessel wall, and the vessel is the center of the lesion. The perivascular tissue can also be involved, but the bronchial central granulomatosis is an exception. Can be involved, can also appear capillary inflammation, inflammation often accompanied by fibrinoid necrosis, intimal hyperplasia and perivascular fibrosis, so pulmonary vasculitis can lead to occlusion of blood vessels and occlusive vascular disease.

Inflammatory cells include neutrophils, normal or abnormal lymphocytes, eosinophils, monocytes, macrophages, histiocytes, plasma cells, and multinucleated giant cells, and many of them are mixed in multiple components, such as neutral. When granulocytes are predominant, it is characterized by leukocyte fragmentation vasculitis; when lymphocytes are predominant, it is the main manifestation of granulomatous vasculitis, but the number of infiltrating inflammatory cells in different vasculitis stages will also There are changes, such as a large number of lymphocytic infiltration after the acute phase of granulocytic vasculitis, but in the late stage of granulomatous vasculitis, inflammatory cells can be mononuclear cells, tissue cells and multinucleated giant cells rather than lymphocytes. .

Although the clinical pathological manifestations of pulmonary vasculitis may be different, there are common immunopathology. In recent decades, anti-neutrophil cytoplasmic antibodies (ANCA) have played an important role in the pathogenesis of vasculitis. effect.

ANCA consists of two broad categories:

1. C-ANCA (cytoplasmic staining pattern of ANCA) is mainly combined with proteinase 3 (PR3) in primary particles of neutrophils, monocytes and immature macrophages. Particle rupture, PR3 release, because of its low charge, so indirect immunofluorescence staining appears as a coarse particle-like intracytoplasmic staining type.

2. P-ANCA (perinuclear staining pattern of ANCA), mainly for the serine proteases in the particles such as myeloperoxidase (MPO), elastase, lactoferrin and other components, these components are more positively charged, indirectly immunized In fluorescent staining, as the rupture of the particles is released, it is easy to bind to the negatively charged nuclei, showing a perinuclear type. P-ANCA is named here. It has been found that C-ANCA against PR3 is in many active Wegener granulations. Swelling patients can be detected in serum and have high specificity. Some patients have positive correlation between C-ANCA titer and disease activity, while P-ANCA for MPO in systemic necrotizing vasculitis including Churg-Strauss syndrome, microscope Lower polyangiitis and idiopathic and drug-induced glomerulonephritis are more common, while atypical P-ANCA for other components are in many diseases such as inflammatory bowel disease, inflammatory liver disease, connective tissue disease, chronic Infection, HIV infection, rheumatoid arthritis can occur, even in a small number of normal people, sometimes ANA can also appear P-ANCA-like staining phenotype in indirect immunofluorescence staining It is mistaken for P-ANCA positive. Therefore, when evaluating the positive result of P-ANCA, it is necessary to analyze the antigen and clinical manifestation. In many cases, atypical P-ANCA only indicates the presence of chronic inflammatory reaction. The diagnosis of vasculitis is not specific.

Most of the ANCA antigens are bactericidal components used by neutrophils in the host defense response, but it is not clear how the immune response will be produced against these autoantigens and the role of the infection in it. Indeed, repeated bacterial infections can lead to blood vessels. Inflammation is aggravated; and vasculitis patients with S. aureus can cause recurrence of vasculitis. There is evidence that cotrimoxazole is effective in treating localized Wegener granulomas and can alleviate recurrence in patients with multiple system involvement.

There are several hypotheses about the pathogenesis of ANCA in vasculitis. One theory is that some pro-inflammatory factors such as IL-1, TGF-, TNF or pathogens can stimulate neutrophils, leading to some component shifts in cytoplasmic granules. Located on the cell surface, it becomes the target of ANCA attack. These cytokines also cause endothelial cells to overexpress adhesion factors. ANCA can also induce neutrophils to release reactive oxygen free radicals and lysosomal enzymes, resulting in local endothelial cell damage. Neutrophils can pass through damaged endothelial cells and accumulate around the blood vessels. Others believe that vascular endothelial cells can express ANCA antigens themselves. In short, ANCA can promote neutrophils to adhere to vascular endothelial cells, indirectly leading to endothelial cell damage. Promote the displacement of neutrophils into the perivascular tissue.

In addition, some studies have shown that the formation and deposition of pathogenic immune complexes is also an important cause of vascular inflammation. Other mechanisms such as endothelial cells are directly infected, and there are anti-endothelial cell antibodies and HLA-dependent T cell-mediated endothelial cells. Cellular damage is also involved in the pathogenesis of vasculitis. Due to differences in endothelial cell response types, different immunopathological mechanisms, and vascular traits, clinically different vasculitis syndromes are caused.

Pulmonary vasculitis prevention

Long-term use of immunosuppressants, need to be alert to lung infections.

Pulmonary vasculitis complications Complications keratitis

Pulmonary vasculitis can be complicated by multiple system and multiple organ damage.

Pulmonary vasculitis symptoms common symptoms abdominal pain atelectasis dyspnea dementia pulmonary interstitial fibrosis vasculitis sensory disorder alveolar hemorrhage

1. Symptoms Pulmonary vasculitis systemic symptoms include fever, fatigue, joint pain and skin lesions, especially in patients with systemic vasculitis and connective tissue disease, granulomatous vasculitis can cause dyspnea and cough, Wegener granuloma And lymphomatoid granuloma, hemoptysis, especially in patients with pulmonary aneurysm or diffuse capillary vasculitis can occur large hemoptysis, churg-Strauss syndrome often accompanied by recurrent dyspnea and asthma history.

2. Various theories

(1) Affecting large, medium and small blood vessels:

1 arteritis: also known as giant cell arteritis, its common clinical manifestations include headache, brachial artery tenderness, myalgia, eye disease, dizziness, dementia and cerebrovascular accident; more common in elderly patients over 60 years old, more common in women, more With anemia and erythrocyte sedimentation rate, it is effective for low-dose corticosteroids. The lymphatic and giant cell infiltration with intimal hyperplasia and elastic layer destruction can be seen in the radial artery biopsy, and the lesions are mostly leaping. The arteritis is often accompanied by rheumatism. Myalgia manifests as fever, fatigue, weight loss, and proximal limb muscle weakness and stiffness. In addition, it has also been reported that arteritis can also affect other blood vessels such as the aorta and pulmonary artery. Therefore, arteritis should be considered systemic. Vasculitis.

2 multiple arteritis: also known as Takayasu disease, mainly involving the aorta and its branches, often causing no pulse, sometimes called no pulse, its pathology is mostly characterized by macrophage infiltration and granuloma formation, causing affected blood vessels Stenosis, occlusion and aneurysm formation, resulting in fever, no pulse, limb pain, abdominal pain, blindness, cerebrovascular accident, hypertension, heart failure and rupture of aneurysm, etc., often accompanied by white blood cells, erythrocyte sedimentation rate C-reactive protein is elevated, and there may be no pulse or bilateral brachial artery pulse strength during physical examination. Vascular murmurs can be heard in the neck or chest and back, and angiography and angiography can further confirm the diagnosis.

Pulmonary artery involvement is more common, reported up to 50%, may be associated with pulmonary hypertension, significant clinical manifestations, such as hemoptysis, chest pain, etc., studies have shown that even in patients without obvious pulmonary symptoms, lung biopsy and angiography There is pulmonary artery involvement.

In the active phase, corticosteroids and immunosuppressive agents such as cyclophosphamide should be given. Arterial stenosis, occlusion and aneurysm formation should be sought for vascular surgical treatment. It is reported that the incidence of tuberculosis infection is high in this country. Infection may be possible, but anti-tuberculosis treatment is not recommended for all patients.

(2) Mainly affecting medium and small blood vessels:

1 Nodular polyarteritis: a systemic disease involving multiple systems. The main pathological manifestations are middle and small myometrial neutrophil infiltration with intimal hyperplasia, fibrinoid necrosis, vascular occlusion and aneurysm formation. Etc., so that the affected tissue ischemic and infarction, the joint muscle, liver and mesenteric vessels, testis, peripheral nervous system and kidney involvement, lung and blood vessels have been affected, and most of the opinions are considered to be nodular. Arteritis rarely affects the lungs, so evidence of pulmonary vascular involvement should be noted with microscopic polyangiitis, Churg-Strauss syndrome and Wegener granulomatosis.

2Churg-Strauss syndrome: also known as allergic granulomatous vasculitis, first named by Churg and Strauss in 1951, hence the name, mainly characterized by asthma, eosinophilia and granulomatous vasculitis A systemic disease in which middle-aged men are more likely to have asthma and asthma symptoms. The lungs, peripheral nerves, heart, and skin are often involved. Foreign reports of kidney involvement are rare and lesions are mild. 11 cases have been reported so far in Beijing. There are 6 cases confirmed by pathology in a hospital in the district. Kidney involvement is not uncommon, reaching more than 50%, and renal insufficiency may occur. Churg-Strauss syndrome may have cough, hemoptysis, chest in addition to asthma. Imaging studies showed flaky infiltrates or nodular shadows. Churg-Strauss did not progress quickly. Active adrenocortical hormones combined with immunosuppressive therapy were effective. Six patients in a hospital in Beijing were actively treated and their conditions were controlled. Renal function returned to normal, and 1 patient died of myocardial infarction after 2 years of drug reduction.

3Wegener granuloma: Wegener was first reported, hence the name, its clinical manifestations are upper and lower respiratory tract necrotizing granulomatous vasculitis and glomerulonephritis, but also involving the eyes, ears, heart, skin, joints and peripheral and central The nervous system, if only the upper and lower respiratory tract are involved without kidney involvement, is called Wegener granulomatosis, the cause is unknown, but Wegener granulomatosis is associated with hypergammaglobulinemia and ANCA positive, and is effective for cytotoxic drugs. Explain that immune mechanisms are involved in the onset

Wegener granuloma can occur in all ages, but it is more common in middle-aged men. The lung lesions can be light and heavy. In severe cases, it can be fatal. Two-thirds of patients can have chest X-ray abnormalities, which can be affected by one side or both sides. Involved, mainly showing pulmonary infiltrates or nodules, some with cavities, bronchial lesions can cause atelectasis, pleural thickening and pleural effusion can also occur, pathological biopsy often manifests as lung tissue necrosis with granuloma Inflammation, infiltrating cells including neutrophils, lymphocytes, plasma cells, eosinophils and tissue cells, vascular inflammation can lead to vascular occlusion and infarction; 1/3 of patients may have hemoptysis in pulmonary capillary vasculitis, in addition, some patients Pulmonary interstitial fibrosis, acute and chronic bronchiolitis, occlusive bronchiolitis and so on.

A large number of clinical studies have shown that ANCA, especially C-ANCA for PR3, although it can be seen in other vasculitis, can occur in more than 80% of Wegener granulomatosis. Continuous high density often indicates disease activity, and with The severity of the disease can also be reduced, which can be used as an important indicator to monitor the activity of Wegener's granulomatosis.

With the application of cytotoxic drugs, especially cyclophosphamide, the mortality of Wegener granuloma has been significantly reduced. The dose of cyclophosphamide is generally 1~2mg/(kg·d), or 2~4mg/(kg·d). It is given once every other day, and it is combined with corticosteroids for at least 1 year. It can be gradually reduced afterwards. It has been reported that it can be maintained by methotrexate. For some patients with infection or easy recurrence, the compound can be added. Nomin, studies have shown that it can reduce recurrence.

(3) Mainly affect small blood vessels:

1 Microscopic polyangiitis: Microscopic polyangiitis is an independent vasculitis that is isolated from nodular polyarteritis. Its clinical manifestations are necrotizing arterioles, venules and capillary inflammation, mainly involving Kidney, skin and lungs, mostly associated with ANCA, no or little immune deposits in the affected vessels, fibrinous necrosis and polymorphonuclear leukocyte and monocyte infiltration, thrombosis, and renal manifestations For focal segmental glomerulonephritis, sometimes with crescent formation; lung involvement is characterized by necrotizing pulmonary capillary vasculitis, and few immunoglobulins and complement deposition.

The disease is common in the elderly, males are slightly more, with fatigue, weight loss, fever, joint pain and other systemic symptoms at the onset, kidney involvement is common, manifested as proteinuria, hematuria or cell cast; many patients show rapid progression Glomerulonephritis, a hospital in Beijing has diagnosed 14 cases of polyangiitis under the microscope, 8 cases of renal insufficiency or even uremia, skin involvement with purpura or nodules, eye, gastrointestinal and peripheral nerves Involved in the lungs, pulmonary infiltrates and hemoptysis, sometimes large hemoptysis, almost all patients with ANCA positive, mainly anti-MPO or anti-PR3 type ANCA, patients with lung involvement are more prone to anti-MPO type ANCA positive, the treatment principle is the same Wegener's granuloma, a 5-year survival rate of about 60%, and more often in the first year, kidney failure and infection are the main cause of death.

2 allergic purpura: also known as Henoch-Schënlein purpura, more common in children, adults can also be affected, is a white blood cell rupture vasculitis, mostly accompanied by upper respiratory tract infection, followed by hip and lower limb purpura, arthritis and abdominal pain Some patients may also have microscopic hematuria and glomerulonephritis. Respiratory involvement is relatively rare. It can manifest as alveolar hemorrhage and flaky infiltration around the hilar. Serum IgA can be elevated. IgA deposition can also be seen in tissue biopsy immunofluorescence.

(4) Baisai syndrome: Baisai syndrome can involve both large blood vessels and small blood vessels; it can involve both arteries and veins, and its clinical manifestations include recurrent oral pain ulcers and perineal ulcers. With arthralgia, nodular erythema or pustular papules, lower extremity venous thrombophlebitis and ocular uveitis, may also affect the digestive tract, cardiovascular, nervous system, kidney and lung, active patients may have positive reaction to acupuncture IgG and complement deposition may occur in the affected area.

10% of patients may have lung involvement, manifested as recurrent pneumonia and hemoptysis, sometimes fatal hemoptysis may occur, hemoptysis may be due to pulmonary vasculitis or bronchial rupture, or may be due to pulmonary aneurysm rupture or arteriovenous fistula As a result, Behcet's syndrome has important organs such as the eye, nervous system, digestive tract, lung involvement should be treated with active immunosuppressive agents, disease activity hemoptysis surgery is not effective, easy to relapse or new aneurysms.

(5) Vasculitis secondary to connective tissue disease:

1 systemic lupus erythematosus: systemic lupus erythematosus lung involvement mainly manifested as pleurisy, pleural effusion, also can occur atelectasis, acute lupus vasculitis, diffuse pulmonary interstitial lesions and vasculitis, etc., pulmonary vasculitis It is a leukocyte fragment vasculitis, which can be accompanied by fibrinoid necrosis, but the specific incidence rate in lupus erythematosus has been reported differently. Some patients may have pulmonary hypertension, mostly light-moderate, some in Beijing. Hospital data indicate severe severe pulmonary hypertension or even right heart failure, and such patients have a poor prognosis.

2 types of rheumatoid arthritis: in addition to joint involvement, vasculitis can also appear, the lung involvement often manifests as pleurisy or pleural effusion, intrapulmonary nodules, interstitial lung disease, some patients may have pulmonary vasculitis and pulmonary artery High pressure, but the incidence is low.

3 systemic sclerosis: the main clinical manifestations are fingertip hardening and skin hardening of the trunk and limbs. Patients are often accompanied by obvious Raynaud's phenomenon and pulmonary interstitial pulmonary hypertension. Intimal hyperplasia of small arteries and arterioles may occur, and centripetal fibrosis may result. Arteriolar stenosis and occlusion, but inflammatory cell infiltration and fibrinoid necrosis are not common, so in a strict sense, it can not be called vasculitis, and most of the treatment of hormones and immunosuppressive agents is ineffective.

4 Sjogren's syndrome: an autoimmune disease mainly caused by epithelial involvement of the exocrine glands. Foreign and domestic epidemiological data indicate that Sjogren's syndrome is not a rare disease. It is called autoimmune epitheliitis because of its opinion. It can not only affect the dry mouth of the salivary glands, but also affect the renal tubular epithelium causing renal tubular acidosis, involving the hepatic bile duct epithelium, pancreatic duct epithelium and gastrointestinal gland epithelium causing gastrointestinal symptoms, involving the lung bronchiole epithelium causing the lungs Fibrosis and pulmonary hypertension.

Sjogren's syndrome vasculitis and hypergammaglobulinemia are also important pathogenic mechanisms of pulmonary interstitial fibrosis and pulmonary hypertension. Treatment emphasizes the treatment of active corticosteroids and immunosuppressive agents in the early stage of pulmonary interstitial lesions.

(6) cryoglobulinemia: recurrent purpura, joint pain and kidney and other internal organs involved, accompanied by increased serum cryoglobulin content and rheumatoid factor positive is the clinical features of this disease, leukocyte invasive vasculitis, blood vessels Immunoglobulin and complement deposition in the wall are histological features, and the lungs can also be invaded by diffuse interstitial infiltration, and the pulmonary vasculature also exhibits the aforementioned inflammatory changes.

(7) Other sporadic pulmonary vasculitis: These diseases are mainly lung-based diseases, and may also have pulmonary vasculitis.

1 Lymphomatoid granulomatosis: It is a blood vessel-centered granulomatosis disease. The lungs are infringed without exception. It was first described by Liebow et al. in 1972. Histomorphology is mainly characterized by upper and lower respiratory tract, skin, central nervous system. Invasive cells are mainly destructive invasive lesions. The infiltrating cells are mainly lymphoblasts, plasma cells, histiocytes, and atypical large lymphocytes containing abnormal mitotic figures, and form granulomatous lesions.

This disease is rare. In 1979, there were 507 reports in the literature. A hospital in Beijing recently reported 3 cases of pathological confirmation. The age of onset was 7-85 years old, with an average age of 48 years. The ratio of male to female was 2:1. Unlike Wegener's granulomatosis, lower respiratory symptoms are more common such as chest pain, difficulty breathing, coughing, etc., but chest X-ray findings are also multiple nodules with shadow formation and cavity formation similar to Wegener granulomatosis, pleural cavity More common fluid, but the hilar lymph nodes rarely invade, the central and peripheral nervous system are often involved in such as cerebral infarction, peripheral neuropathy, etc., the kidney is rarely involved, laboratory tests are often difficult to help diagnosis, skin lesion biopsy may help It depends on pathological examination to determine the diagnosis.

Untreated lymphoma-like granuloma usually deteriorates rapidly and eventually dies in central nervous system lesions. About half of the patients may be relieved by cyclophosphamide and corticosteroid treatment. The average survival time is 4 years. When the treatment cannot be relieved, it will develop into Vascular central T-cell lymphoma, but there may be benign types, the latter mainly manifested by vasculitis and granuloma formation of pleomorphic lymphocytic infiltration, few tissue necrosis, good treatment response, also known as For "lymphocytic vasculitis and granulomatosis".

2 necrotic sarcoidosis-like granulomatosis: first reported by Liebow in 1973, histological features of granulomatous lesions in the lung, similar in morphology and sarcoidosis, but with necrotic granulation of pulmonary artery and vein Tumor vasculitis lesions, about half of patients without hilar lymphadenopathy, and typical sarcoidosis, the disease has a good prognosis, often natural relief, may be a variant of sarcoidosis.

3 bronchial central granulomatosis: clinical symptoms may have fever, fatigue, cough, asthma, etc., eosinophil count can be increased, chest X-ray film shows invasive or nodular shadow, can also occur atelectasis, and Other systemic vasculitis diseases are different from multiple organ involvement, and half of the patients are related to Aspergillus or other fungi; the lungs are centered on the bronchi, infiltrated by lymphocytes and plasma cells to destroy small airways, and granuloma formation is basic. Histological changes, small arteries and veins near the lesion can be violated, so pulmonary vasculitis is a secondary pathological process, the prognosis is better, can be naturally relieved, only symptomatic treatment, severe symptoms require corticosteroid treatment.

3. Signs of signs and organs involved, such as leukocyte fragment vasculitis, rash and ulcers are more obvious, joint deformation suggests rheumatoid arthritis, nasal and upper respiratory ulcers suggest that there may be wegener granulomatosis or lymphoma-like granulation Swelling, the former may also have ptosis and keratitis, uveitis, Behcet's disease with oral cavity, perineal ulcer and uveitis, nodular polyarteritis and Churg-Strauss syndrome often appear peripheral nerve Involved, while giant cell arteritis may have signs of central nervous system involvement, and the signs of the lungs vary depending on the degree of pathological invasion.

Pulmonary vasculitis

Often there are positive cell anemia, thrombocytosis, increased polyclonal r-globulin, decreased albumin levels, increased ESR, increased CRP, and abnormal liver enzymes, all of which suggest an acute phase reaction.

Angiography showed: irregular lumen, stenosis and occlusion of the lumen, tumor-like dilatation, ultrasound diagnosis of cardiovascular disease, and thickening of the vessel wall, stenosis and other lesions in imaging, imaging X-ray tomography can also find lesions such as thickening of the vessel wall and stenosis of the lumen. Magnetic resonance examination in imaging can also reveal lesions such as thickening of the vessel wall and stenosis of the lumen.

Diagnosis and diagnosis of pulmonary vasculitis

Diagnostic criteria

In all vasculitis, some skin lesions, systemic and muscular joint symptoms appear, and some inflammatory response indicators are abnormal in laboratory tests. These abnormalities should be noted to exclude vasculitis. Systemic symptoms of vasculitis include: fever. Anorexia, weight loss, fatigue, etc., muscle and joint symptoms include: rheumatic polymyalgia-like symptoms, joint pain or arthritis, myalgia and peripheral neuropathy, laboratory tests often show positive cell anemia, thrombocytosis, albumin Decreased levels, increased polyclonal globulin, increased ESR, increased CRP, and abnormal liver enzymes, all suggest an acute phase reaction of inflammation. To diagnose vasculitis, first of all, have a full understanding of the clinical manifestations of different vasculitis, combined with specific patients Diagnosis of clinical, laboratory, histopathological or angiographic abnormalities, and attention to differential diagnosis with some secondary vasculitis.

The diagnosis of vasculitis requires biopsy or angiography. These tests should be performed as much as possible to confirm the diagnosis of vasculitis. Once vasculitis is diagnosed, long-term treatment is needed, and the toxic side effects of the treatment are more. Table 1 lists the common vasculitis diagnosis. Biopsy site and angiographic sensitivity, but this sensitivity varies among different investigators and different study populations.

In general, biopsy should be performed on symptomatic and convenient parts. The positive rate of blind detection on asymptomatic parts such as muscle, testis or nerve is low, and the skin, muscle, nasal mucosa and radial artery biopsy are well tolerated. Easy to obtain, although it is not specific for the diagnosis of a vasculitis skin biopsy, combined with clinical, laboratory and radiological findings, often can diagnose vasculitis, testicular involvement is rare, and testicular biopsy requires general anesthesia, patients Sometimes it is difficult to accept. If the patient has clinical manifestations of neuropathy or abnormal measurement of EMG and nerve conduction velocity, it is helpful to perform a sural nerve biopsy. However, biopsy often has a sequela of local sensory disturbance in the distal extremity. Percutaneous renal biopsy is not Dangerous, but vasculitis is not much, the most common histopathological manifestations of focal segmental necrotizing glomerulonephritis, for the diagnosis of pulmonary vasculitis, the positive rate of transbronchial lung biopsy is not high, should be open chest biopsy Or thoracoscopic lung biopsy.

For suspected vasculitis, but there is no suitable biopsy site, angiography should be performed. Vasculitis angiography is usually characterized by segmental arterial stenosis, sometimes cystic aneurysm-like dilatation and occlusion, usually using abdominal angiography, sometimes despite Abdominal angiography can also be abnormal, and abnormalities can occur in the kidney, liver and mesenteric vessels. The angiographic cystic aneurysm is more serious, and effective treatment can reverse angiographic abnormalities, but the angiographic specificity is not High, many vasculitis and secondary vasculitis can cause similar angiographic abnormalities, such as nodular polyarteritis, Wegener's granulomatosis, Churg-Strauss syndrome, rheumatoid arthritis and systemic lupus erythematosus Inflammation and Behcet's syndrome, in addition, other diseases, such as left atrial myxoma, bacterial endocarditis, thrombotic thrombocytopenic purpura, abdominal tuberculosis, arterial dissection, tumor, pancreatitis, etc. can cause angiography Abnormal, in giant cell arteritis, aortitis, Buerger disease, its angiography has certain characteristics, affected blood vessels Different cloth and no aneurysm sac-like performance.

Differential diagnosis

1. Infectious vasculitis Many different pathogen infections can cause vasculitis-like manifestations, including bacteria (such as streptococci, staphylococcus, salmonella, Yersin's disease, mycobacteria, Pseudomonas, etc.), fungi, Keploid, Borrelia burgdorferi and viral infections (such as hepatitis A, B, hepatitis C virus, cytomegalovirus, Epstein-Barr virus, herpes zoster virus, HIV virus, etc.), according to their clinical manifestations and corresponding laboratory tests are mostly easy Identification, hypersensitivity vasculitis caused by infectious diseases is mainly caused by skin lesions.

2. Tumor or connective tissue disease secondary vasculitis When patients have vasculitis-like manifestations (especially with skin lesions), if accompanied by hepatosplenomegaly, lymphadenopathy, decreased cells or abnormal peripheral blood smears Attention should be paid to the exclusion of vasculitis secondary to tumors. Lymphoma, leukemia and reticuloendothelial system are prone to this manifestation, while solid tumors are relatively rare. In addition, some connective tissue diseases may also have secondary vasculitis. Performance, common are: rheumatoid arthritis, Sjogren's syndrome and systemic lupus erythematosus, need to pay attention to identify.

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