Secondary systemic amyloidosis
Introduction
Introduction to secondary systemic amyloidosis Secondary systemic amyloidosis refers to systemic amyloidosis secondary to certain pathologies such as chronic inflammation, chronic local or systemic bacterial infections, malignancies, and the like. basic knowledge Sickness ratio: 0.0012% Susceptible people: no special people Mode of infection: non-infectious Complications: proteinuria uremia urticaria purpura nodular vasculitis
Cause
Secondary systemic amyloidosis
Chronic inflammation (45%):
Includes rheumatoid arthritis, juvenile chronic arthritis, ankylosing spondylitis, psoriasis (vulgaris and joint disease), Reiter syndrome, adult Still disease, Behcet syndrome, Crohn's disease, chronic suppurative dermatosis Wait.
Chronic bacterial infections (25%):
Including leprosy, tuberculosis, bronchiectasis, hemorrhoid ulcer, osteomyelitis, Whipple disease, syphilis, suppurative sweat gland inflammation, chronic pyelonephritis in paraplegic patients.
Malignant tumor (8%):
Including Hodgkin's disease, hairy cell leukemia, kidney cancer, intestinal tract, lung and genitourinary cancer, basal cell carcinoma and the like.
Pathogenesis
The amyloid protein of this disease is amyloid A proterin (AA protein), which is not related to immunoglobulin. Its precursor is called serum amyloid A-related protein (SAA protein), which is normal. A small amount exists in human serum, and the AA protein constitutes the amino terminal part of the SAA protein. The latter is proteolytically produced in the macrophage lysosome to produce AA protein, which is stimulated by antigens in various primary macrophages to secrete AA protein. It has been deposited outside the cell and has been diagnosed. In recent years, it has been found that certain endocrine tumors produce amyloid and insulin-like amyloid, and glucagon amyloid, which are associated with pancreatic diseases, belong to the AA protein. Some scholars have reported multiple Endocrine neoplasia type 2A (MEN 2A) is associated with back pain, paresthesia, local pruritic pigmentary plaques and amyloidosis of the skin. Amyloid protein is derived from keratin, the so-called amyloid K protein. Immunohistochemistry, electron microscopy and immunoelectron microscopy revealed that the basement membrane of the lesion was destroyed, and the dense plate structure was destroyed and amyloid deposition was observed, suggesting true - Interface skin damage.
Prevention
Secondary systemic amyloidosis prevention
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Secondary systemic amyloid complication Complications, proteinuria, urticaria, urticaria, nodular vasculitis
The disease mainly invades the liver, spleen, kidney, adrenal gland and skin and other organs, manifested as hepatosplenomegaly, proteinuria, secondary nephritis and uremia. Skin manifestations include calf erysipelas-like damage, urticaria, Schonlein-Henoch purpura, nodular vasculitis, etc.
Diagnosis is based on multiple organ damage, pathological amyloid deposition, and the like.
Symptom
Secondary systemic amyloidosis symptoms common symptoms proteinuria hepatosplenomegaly nodule amyloid deposition
The disease mainly invades the liver, spleen, kidney, adrenal gland and skin and other organs, manifested as hepatosplenomegaly, proteinuria, secondary nephritis and uremia, skin manifestations of calf erysipelas, urticaria, Schonlein-Henoch purpura, Nodular vasculitis and the like.
Examine
Secondary systemic amyloidosis
Different test abnormalities may occur in different organs, such as abnormal liver function, proteinuria, and azotemia.
Histopathology: amyloid deposits in the interstitial tissues and blood vessel walls of the affected organs. The long-term parenchymal cells are replaced by amyloid. Skin biopsy should include subcutaneous fat layer, amyloid deposits in the lower dermis or adipose tissue, attached. Around the small blood vessels.
Diagnosis
Diagnosis of secondary systemic amyloidosis
Diagnosis is based on multiple organ damage, pathological amyloid deposition, and the like.
Care should be taken to identify the primary disease and identify it with primary systemic amyloidosis.
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