The symptoms of this rare “strange disease” vary, and it also specifically targets middle-aged and elderly men
The Metropolitan Express reported that not long ago, three men were diagnosed in the Nephrology Department of Run Run Shaw Hospital Affiliated to Zhejiang University School of Medicine. For the same rare disease, but their symptoms are very different.
The high globulin was found in the physical examination four years ago
I don’t take it seriously if I am not feeling well
The urine protein was positive due to the physical examination, 39 years old Mr. He came to the Nephrology Clinic of Run Run Run Run Shaw Hospital of Zhejiang University. Deputy Chief Physician Xiong Xiaoling read Mr. He’s checklist. In addition to “urinary protein 2+”, he found that serum globulin was more than three times the upper limit of normal. cause alertness.
Elevated levels of globulin often indicate possible liver disease, chronic inflammation, or autoimmune disease in the body. In clinical, many diseases can have elevated globulin, such as viral hepatitis, tuberculosis, lymphoma and so on. But such high globulin is rare.
Mr. He said that during his physical examination four years ago, he found that his globulin was too high. In recent years, the number of regular physical examinations has been getting higher and higher. Because no other faults have been found, he has been indifferent. Of.
In addition, Mr. He pointed to his upper eyelids, “A few years ago, I had a lump on each side of my eyelids inexplicably. It was neither painful nor itchy, but this lump was pressing down. , I always feel that I can’t lift my eyelids, and I look listless.”
“But, does this have anything to do with my urine protein?” Mr. He was a little puzzled.
One month ago, the kidneys were still good
The 69-year-old uncle is at risk of kidney failure
The 69-year-old Uncle Shi is also puzzled. He couldn’t figure it out, his kidneys were fine a month ago, but in just one month, he was at risk of kidney failure. He came to the Shaw Hospital of Zhejiang University because of nausea and vomiting. He sat in the emergency room and was a little dazed at the critical value of “blood muscle 635μmol/L↑↑” on the test sheet.
In order to find out the cause of acute renal failure as soon as possible, Uncle Shi was admitted to the Nephrology Department of Run Run Run Shaw Hospital and completed a series of examinations. A few days later, the results of a paper abdominal CT seemed to lift the veil of the disease: the retroperitoneal band shadow with its upper double urethra, retroperitoneal fibrosis should be considered.
The cause of Shi Dabo’s acute renal failure has been explained – the occurrence of hydronephrosis confirmed the existence of urinary tract obstruction, which is one of the common causes of clinical acute renal failure.
But what’s behind urinary obstruction and retroperitoneal fibrosis?
63-year-old Wu was re-admitted to the hospital due to yellow skin
The results of abdominal CT made my heart sink to the bottom
Compared with Mr. He and Uncle Shi, 63 The old Wu’s condition seems to be much clearer. Since being diagnosed with diabetes three years ago, Wu feels that he has been in constant trouble and has a bad fate. First, two years ago, because of “ureteral stenosis; ureteral wall thickening, suspicious space occupation”, the operation of ureteral dilatation was performed.
A year ago, Wu was re-admitted to the hospital due to yellow skin. The results of abdominal CT after admission made the family’s heart sink to the bottom again, “pancreatic mass: pancreatic head cancer with dilation of extrahepatic bile duct and common bile duct”.
The team of Director Cao Qian of the Department of Gastroenterology of Run Run Run Run Shaw Hospital carefully performed a pancreatic puncture for Lao Wu. Combined with blood test, CT scan and pathology, the final diagnosis was autoimmune pancreatitis with bile duct involvement.
Old Wu has been insisting on taking medication and having regular outpatient check-ups, but just four months ago, during the process of gradually reducing the dosage of the drug, Lao Wu experienced a decline in renal function again, and his serum creatinine progressively increased to 50%. 227 μmol/L (normally no more than 110 μmol/L), and urinary protein appeared 2+.
Are kidney damage and autoimmune pancreatitis isolated? Are there close connections between them?
The three patients have different clinical manifestations
But in fact they have the same disease
In fact, these three patients seem to be completely different, with different clinical manifestations , but they all had the same disease-IgG4-related disease, including autoimmune pancreatitis previously diagnosed by Lao Wu, which is also one of the subtypes of this type of disease.
IgG4-related disease is an immune-mediated chronic, progressive inflammatory disease with fibrosis, characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells into tissues and organs. IgG4-RD was only recognized by people in 2003. It is a very rare clinical disease and was included in the “First Batch of Rare Diseases List” in my country.
These diseases are more common in middle-aged and elderly men. Although the etiology and pathogenesis are still unclear, as a systemic disease involving multiple organs, IgG4-RD can affect almost all parts of the body. Hormones are currently recognized as the first choice for the treatment of IgG4-RD.
The three patients mentioned above all received hormones or hormones combined with immunosuppressive therapy, and achieved good results. Mr. He’s globulin decreased significantly, the orbital mass disappeared, and proteinuria also decreased; after Mr. Shi’s treatment, his serum creatinine dropped rapidly, his renal function was saved, and urinary intubation and hemodialysis were avoided; Lao Wu’s renal function was significantly improved , the urine protein disappeared, and the lesions of the pancreas and bile duct were also stably controlled.
However, it should be noted that although the treatment response is good, the disease is still a chronic disease that is difficult to cure, and the disease may recur during the process of induction therapy or drug dose reduction. Therefore, patients entering maintenance therapy should not be taken lightly, and they need to be followed closely under the guidance of doctors.