They have cured 2 consecutive cases of this rare disease that is specially selected for middle-aged and elderly men!

Mr. Tang, a 57-year-old from Yongzhou, Hunan, has suffered from upper abdominal distension and pain in the past six months, and his skin, eyes and The color of urine turned yellow, and the stool was close to clay color, which was suspected to be malignant tumor of hilar bile duct by many hospitals. After being hospitalized for injections, medicines, and puncture and drainage, the symptoms eased slightly; but each time he was discharged from the hospital, the above-mentioned symptoms would come back again, causing him to lose nearly 20 kilograms in just half a year, and he was almost desperate.

Introduced by someone, Mr. Tang was admitted to the Fifth Hepatobiliary Ward of Hunan Provincial People’s Hospital on May 13, 2022. Under the guidance of Chief Physician Wang Xintian, the Director of the Ward, the team of Chief Physician Chen Chen, Deputy Director of the Ward, discussed the condition, improved relevant examinations, and invited multidisciplinary experts for consultation. The pathological result obtained was “IgG4-related chronic sclerosing cholangitis”. An extremely rare disease.

After the diagnosis was made, Director Chen Chen’s team provided timely symptomatic treatment to Mr. Tang, and Head Nurse Hu Xiahong led the nursing team to customize and implement a rapid recovery plan for him. Mr. Tang’s symptoms quickly eased and he recovered and was discharged from the hospital on May 30.

Coincidentally, Mr. Xiao, 60, from Yiyang, Hunan, is also a patient with IgG4-related disease. Since the beginning of April this year, he found that his skin and eyes were getting more and more yellow, he also had itchy skin all over his body, and his appetite was much worse. After visiting many hospitals, he was suspected of obstructive jaundice caused by bile duct cancer or pancreatic cancer, and he was advised to undergo surgery and chemotherapy.

Mr. Xiao couldn’t accept this result, so he went to the Department of Hepatobiliary Surgery of Hunan Provincial People’s Hospital for treatment, and then he was admitted to the fifth hepatobiliary ward. After a multidisciplinary discussion with the nephrology and radiology experts, the team of chief physician Chen Chen quickly identified the “true culprit” – IgG4-related chronic sclerosing cholangitis, and immediately transferred him to the second kidney in the kidney. The ward receives symptomatic treatment.

After 2 weeks of treatment, Mr. Xiao’s jaundice basically subsided and he was discharged from the hospital on June 8th. Before leaving the hospital, he deliberately returned to the fifth hepatobiliary ward and presented a pennant to the team of chief physician Chen Chen to express his gratitude.

“IgG4-related disease is a chronic inflammatory autoimmune Elderly males, with an average age of onset >50 years old. Mainly manifested as swelling of the involved organs or inflammatory pseudotumor formation, characterized by elevated IgG4 levels and rich in lymphoplasmacytic cells that mainly secrete IgG4 in the involved tissues,” said Chief Physician Chen Chen. , IgG4-related diseases were only included in the “First Batch of Rare Disease Catalogues” jointly announced by the National Health Commission and other five departments in May 2018.However, IgG4-related sclerosing cholangitis is a manifestation of the biliary system of IgG4-related diseases. Currently, less than 200 cases have been reported in domestic literature. Because it is rarely encountered in daily diagnosis and treatment, many doctors do not have a deep understanding of this rare disease. In addition, the disease involves a wide range, and patients often go to different departments for treatment with the first symptoms. It may be misdiagnosed or missed, causing unnecessary pain to patients. Therefore, the precise diagnosis and treatment of IgG4-related diseases requires not only a pair of “wisdom eyes” of clinicians to identify the disease, but also the participation of a multidisciplinary diagnosis and treatment model (MDT).

In recent years, MDT has become a new development trend in clinical diagnosis and treatment. The Fifth Hepatobiliary Ward of Hunan Provincial People’s Hospital treats a large number of patients with intractable biliary tract diseases every year. After multidisciplinary joint diagnosis and treatment, satisfactory results have been obtained, which is a rare disease. And difficult and critically ill patients bring good news.