(1) Clinical manifestations

L. Kidney

(1) Proteinuria: it is the main feature of the early stage of this disease and can be used as the only manifestation of renal damage, which lasts for several years. Macromolecular proteinuria is the main cause, and the degree of proteinuria varies (+~+++).

(2) Nephrotic syndrome: Most of the onset is hidden, and once it appears, the disease progresses rapidly.

(3) Renal insufficiency: progressive renal dysfunction, and severe cases died of uremia.

(4) Renal interstitial tubulointerstitial lesion: ① Significantly polyuria, with a daily urine output of 3 ~ 6L, and even diabetes insipidus, with hypotonic urine after hypertonic saline and vasopressin. ② Glucose urine. ③ Phosphate excretion increased. ④ Renal tubular acidosis. ⑤ electrolyte disorder.

2. Extrarenal organs

(1) Primary amyloidosis: ① heart failure and arrhythmia. ② Gastrointestinal bleeding and intestinal obstruction. ③ paresthesia of extremities, decreased muscle tone and carpal tunnel syndrome. ④ Orthostatic hypotension. In addition, there are corresponding manifestations of bone marrow, smooth muscle, bone and joint involvement.

(2) Secondary renal amyloidosis: liver, spleen and adrenal gland are the main organs involved.

(3) Associated with multiple myeloma: bone pain is the main symptom.

(4) Amyloidosis in the elderly: it mostly occurs in brain, heart, pancreas, aorta and bone and joint tissues.

(5) Long-term hemodialysis patients: the amyloidosis caused by β2-m accumulation, manifested as carpal tunnel syndrome, arthritis, pathological fracture, and gastrointestinal, heart, liver, spleen, lung and testis involvement.

(2) Laboratory examination

1. Urine was positive for Bence-Jone protein.

2. The positive rate of peak globulin per plant determined by electrophoresis was almost 1% in primary amyloidosis and 53% in secondary amyloidosis.

3. Blood biochemical examination showed that erythrocyte sedimentation rate increased, fibrinogen decreased and fibrinolysis increased.

4. BUN and Scr increased in the late stage of renal function.

(3) Special examination

1. Ultrasound, KuB and IVP showed that the volume of both kidneys increased, especially when renal failure occurred.

2. Biopsy of skin, mucosa and kidney tissue is the most reliable method for diagnosis, and the positive rate is 75% ~ 85%.

(4) Diagnostic criteria

1. Chronic suppurative inflammation, tuberculosis or rheumatoid arthritis complicated with nephrotic syndrome.

2. Kidney disease complicated with cardiomyopathy, neuropathy and giant tongue.

3. Renal volume does not shrink when renal failure occurs.

4. Urine was positive for Bence-Jone protein.

5. Biopsy was positive.

Senile nephrotic syndrome (or proteinuria) and Fanconi syndrome with positive urine Bence-Jone protein should be suspected.