Benign lymphocytic vasculitis and granulomatous disease of the lung

Brief introduction of benign lymphocytic vasculitis and granulomatosis Benign lymphoblastic vasculitis and granulomatosis (BLAG) was proposed by Saldana et al in 1977. It is a disease similar to Wegener's granulomatous and lymphomatoid granulomatosis. The lesions are found in the skin and respiratory tract. Mainly manifested as chronic recurrent panniculitis. basic knowledge The proportion of illness: 0.005%--0.007% Susceptible people: more common in men Mode of infection: non-infectious Complications: myocardial infarction

Benign lymphocytic vasculitis and granulomatosis

(1) Causes of the disease

The cause is unknown.

(two) pathogenesis

Pathological examination showed that the alveolar structure disappeared, and there were granuloma with unclear borders. The vasculitis was mainly caused by invasive damage of blood vessels and invading blood vessels. Infiltrating cells had normal lymphocytes, plasma cells, histiocytes, and atypical lymphocytes. The morphology of plasma cells, reticulocytes, and morphological cells are mitotic, similar to lymphoproliferative diseases. Occasionally large lymphocytes, some small blood vessels may cause partial embolism and necrosis of the lungs due to obstruction after lymphocyte infiltration. , but such situations are rare.

Benign lymphocytic vasculitis and granulomatosis prevention

1. Age > 60 years old and those with moderate or moderate renal function, the dose of cyclophosphamide decreased to 25 mg / d.

2. If the total number of white blood cells <4.0×109/L or neutrophils<2.0×109/L, the immunosuppressant is immediately stopped.

3. The amount of corticosteroids to be used should be large. When using it, it can be replaced with a regular preparation or a high-low dose during the day.

Benign lymphocytic vasculitis and granulomatosis complications Complications, myocardial infarction

Can block blood vessels causing inflammation, infarction and necrosis.

Pulmonary benign lymphocytic vasculitis and granulomatosis symptoms Common symptoms Weak hemoptysis granuloma serum immunoglobulin can increase chest tightness and lymphatic hyperplasia

The patient has no specific clinical manifestations, initial systemic fatigue and discomfort, followed by fever, cough, chest tightness and other symptoms. Hemoptysis is rarer than other granulomatous diseases. 80% of patients in the chest radiograph have multiple nodules of different sizes in the lungs. Shadow, about 20% of patients with chest radiographs are isolated nodules or invasive lesions, some patients can see hollow changes; patients with erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and serum immunoglobulin can be elevated, individual patients in the blood Anti-neutrophil cytoplasmic antibody (ANCA) positive, antibody type similar to Wegener granulomatosis, belonging to C-ANCA.

The disease is more common in men, the age of onset is 31 to 74 years old, the average is 57 years old, except for skin lesions, extrapulmonary invasion is rare, the patient has no specific clinical manifestations, early systemic fatigue and discomfort, followed by fever, cough, Symptoms such as chest tightness, hemoptysis is rare compared with other granulomatous diseases. 80% of patients in chest radiographs have multiple nodular shadows of different sizes in the lungs. About 20% of patients have isolated nodules or invasive lesions, some patients Visible cavity changes; patient erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and serum immunoglobulin can be elevated, individual patients with anti-neutrophil cytoplasmic antibody (ANCA) positive, antibody type similar to Wegener granulomatosis , belongs to C-ANCA.

The diagnosis of BLAG depends on lung biopsy. The pathological examination shows that the alveolar structure disappears, and there is a granuloma with unclear borders. The vasculitis is mainly caused by invasive damage of blood vessels and blood cells invading the blood vessels. The infiltrating cells have normal lymphocytes and plasma. Cells, tissue cells, atypical lymphocyte-like and plasmacytoid cells, reticuloendothelial cells, and morphological changes of morphological cells are the same as lymphoproliferative diseases. Occasionally, large lymphocytes may be infiltrated by lymphocytes after infiltration. Obstruction causes local embolism and necrosis of the lungs, but such cases are rare. The disease needs to be differentiated from lymphoma-like granuloma, Wegener granulomatosis, and lack of necrosis is the main distinguishing point between this disease and Wegener granulomatosis.

Examination of benign lymphocytic vasculitis and granulomatosis

ESR is elevated, C-reactive protein (CRP) is elevated, serum immunoglobulin is elevated, and anti-neutrophil cytoplasmic antibody (ANCA) is positive in individual patients. Antibody type is similar to Wegener granulomatosis. , belongs to C-ANCA.

In 80% of patients with chest radiographs, multiple nodular shadows of different sizes can be seen in the lungs. About 20% of patients have isolated nodules or invasive lesions, and some patients can see hollow changes.

Diagnosis and differentiation of benign lymphocytic vasculitis and granulomatosis

The disease needs to be differentiated from lymphoma-like granuloma, Wegener granulomatosis, etc. The lack of necrotic changes is the main distinguishing point between this disease and Wegener granulomatosis.

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