OVERVIEW
OVERVIEW
ANCA-associated small vessel vasculitis renal injury is the most common type of primary small vessel vasculitis in adults, and clinical manifestations of renal involvement such as hematuria, proteinuria, and acute and chronic renal insufficiency may occur.
Are you insured?
Yes, it is
Department
Nephrology
Alias
ANCA-associated small vessel vasculitis with renal damage
Clinical symptoms
1. Renal manifestations include hematuria, proteinuria, and tubular urine in the active stage; hematuria may disappear in the remission stage. Renal hypoplasia is common.2. Extra-renal manifestations include malaise, fever, weight loss, myalgia, and skin rash.
Hazards
This disease may present symptoms of renal damage such as hematuria, proteinuria, tubular urine, renal hypoplasia, etc. It can often involve multiple organs and present corresponding symptoms and complications, coupled with the fact that patients tend to be old and in poor physical condition, which may be life-threatening.
Complications
Pulmonary hemorrhage, pulmonary infection, hypoxemia, focal segmental glomerulonephritis, etc.
Examination
Urine routine, urine sediment, blood routine, renal function, blood urea nitrogen, creatinine, electrolytes, ANCA-related tests, anti-glomerular basement membrane antibody, ultrasound, renal histopathologic examination, etc.
Diagnosis
Diagnosis is made by combining ANCA-related examination, renal histopathologic examination and other relevant auxiliary examinations with clinical manifestations of systemic multi-system involvement.
Treatment principle
Symptomatic supportive therapy, immunosuppressive therapy, complication prevention and treatment.
Curability
Hormone plus cyclophosphamide treatment can lead to remission in more than 90% of patients.
Dietary advice
If there is edema and hypertension, low-salt diet should be given; protein intake should be limited in azotemia; avoid excessive potassium-containing food when urine is low.
Etiology
Epidemiology
Most patients are middle-aged or elderly, with more men than women.
Etiology
The etiology is unclear, probably related to genetic factors, while infections, drugs and occupational exposure are important predisposing factors. The pathogenesis is mainly due to the involvement of ANCA, T-cells and other immunoreactive cells, as well as cytokines secreted by them, in the damage to the vessel wall.
Symptoms and Diagnosis
Typical Symptoms
The main symptoms manifest as hematuria, proteinuria, and tubular urine. Almost all patients have hematuria, and there is even significant hematuria of the naked eye.
Other symptoms
Systemic nonspecific symptoms such as fever, arthralgia, myalgia, malaise, rash, lack of appetite, and weight loss may be present in the early stage. Other organ involvement manifestations (1) Skin: purpura, rash, ulcers; (2) Lungs: cough, sputum, coughing up blood, chest pain, dyspnea (3) Upper respiratory tract infections: nasal congestion, pain in the sinus area, bloody or purulent discharge from the nasal passages, tinnitus, deafness, perforated eardrums; (4) Eyes: conjunctivitis, corneal ulcers, scleritis; (5) Neurological system: weakness, numbness of the limbs, pain, stupor, cerebral membrane irritation sign; (6) digestive system: nausea, vomiting, abdominal pain, diarrhea, blood in stool, etc.
Diagnostic basis
Patients present with symptoms of renal injury such as hematuria, proteinuria, tubular urine, renal hypoplasia, and other clinical manifestations of multi-organ involvement.Positive ANCA test, positive cANCA and anti-PR3 antibody, positive pANCA combined with positive anti-MPO antibody are highly specific for the diagnosis of ANCA-associated small-vessel vasculitis.Patchy infiltrating shadows, nodules, or cavities can be seen in lungs on chest X-ray or CT examination; Renal histopathology showing focal segmental necrotizing glomerulonephritis or granulomatous vasculitis can confirm the diagnosis.
Treatment
Treatment guidelines
Symptomatic supportive therapy, antihypertensive therapy, remission-inducing drug therapy, and prevention of complications.
Drug therapy
Glucocorticoid oral prednisone is the first choice of drug.2. Cyclophosphamide hormone combined with cyclophosphamide can obtain higher remission rate and lower recurrence rate.3. Diuretics commonly used diuretics such as thiazides, furosemide, etc. are taken orally or intravenously, and vascular antispasmodic drugs, such as small-dose dopamine, can be added to strengthen the effect of diuresis.4. Antihypertensive drugs such as calcium channel blockers and alpha receptor blockers.
Other treatments
Patients with renal failure should be given dialysis treatment promptly, and renal transplantation can be considered for patients with end-stage renal failure.
Prognosis
Without treatment, most patients progress to irreversible renal failure, with a mortality rate of up to 80% within one year, often due to uremia or massive pulmonary hemorrhage. Hormone and cyclophosphamide therapy can lead to remission in more than 90% of patients. Overseas studies have shown that the 8-year survival rate can reach more than 80%.
Nursing care
Daily care
Avoid cold and flu, pay attention to rest, avoid using drugs that can cause kidney damage. Pay attention to monitoring the urine volume, blood pressure, regular recheck urine routine, kidney function, ANCA.
Dietary modification
Those with edema and hypertension should be given low-salt diet; protein intake should be limited in azotemia; avoid excessive potassium-containing food when urinary intake is low.