In amyloidosis, abnormal proteins in the body change shape and clump together to form amyloid fibrils. Those fibrils build up in tissues and organs, which can stop them from working properly.
淀粉样变性是淀粉样变性的最常见类型之一。它也被称为经胆囊素淀粉样变性。它涉及一种称为经甲状腺素蛋白（TTR）的蛋白质，该蛋白在肝脏中产生。
In people with ATTR amyloidosis, TTR forms clumps that may build up in the nerves, heart, or other parts of the body. This can cause potentially life threatening organ failure.
Read on to learn how this condition may affect a person’s life expectancy and the factors that influence survival rates, along with background information about the different types of ATTR amyloidosis and how they’re treated.
Life expectancy and survival rates vary based on the type of ATTR amyloidosis an individual has. The two main types are familial and wild-type.
平均而言，患有家族性淀粉样蛋白病的人活着7至12年根据遗传和稀有疾病信息中心，他们得到诊断后。
A study published in the journal Circulation found that people with wild-type ATTR amyloidosis live an average of大约4年after diagnosis. The 5-year survival rate among study participants was 36 percent.
ATTR amyloidosis often causes amyloid fibrils to build up in the heart. This can cause abnormal heart rhythms and life threatening heart failure.
There’s no known cure for ATTR amyloidosis. However, early diagnosis and treatment may help slow the development of the disease.
几个因素可能会影响患有淀粉样蛋白病的人的生存率和预期寿命，包括：
- the type of ATTR amyloidosis they have
- which organs are affected
- 当他们的症状开始时
- 他们如何开始治疗
- which treatments they receive
- their overall health
More research is needed to learn how different treatment approaches may affect survival rates and life expectancy in people with this condition.
一个人拥有的ATTR淀粉样变性类型将影响其长期前景。
If you’re living with ATTR amyloidosis, but you’re not sure which type, ask your doctor. The two main types are familial and wild-type.
当TTR结块以外的蛋白质成淀粉样蛋白原纤维以外，其他类型的淀粉样变性也可能发展。
Familial ATTR amyloidosis
Familial ATTR amyloidosis is also known as hereditary ATTR amyloidosis. It’s caused by genetic mutations that may be passed from parent to child.
这些遗传突变导致TTR不如正常人稳定。这增加了TTR形成淀粉样蛋白原纤维的机会。
许多不同的遗传突变可能导致家族性淀粉样变性。根据一个人的特定遗传突变，这种情况可能会影响他们的神经，心脏或两者兼而有之。
家族性淀粉样变性的症状从成年开始，随着时间的流逝而变得更糟。
野生型ATTR淀粉样变
野生型ATTR淀粉样变isn’t caused by any known genetic mutations. Instead, it develops as a result of aging processes.
In this type of ATTR amyloidosis, TTR becomes less stable with age and begins to form amyloid fibrils. Those fibrils are most commonly deposited in the heart.
This type of ATTR amyloidosis typically affects men over the age of70 years old。
其他类型的淀粉样变性
Several other types of amyloidosis also exist, including AL and AA amyloidosis. These types involve different proteins than ATTR amyloidosis.
Al淀粉样变性也被称为原发性淀粉样变性。它涉及异常的抗体成分，称为轻链。
AA淀粉样变性也称为继发性淀粉样变性。它涉及一种称为血清淀粉样蛋白的蛋白质。它通常是由感染或炎症性疾病（例如类风湿关节炎）触发的。
If you have ATTR amyloidosis, your doctor’s recommended treatment plan will depend on the specific type you have, as well as the organs that are affected and the symptoms that develop.
根据您的诊断，它们可能会开出以下一项或多项：
- a liver transplant, which is used to treat some cases of familial ATTR amyloidosis
- ATTR silencers, a class of medications that help reduce the production of TTR in people with familial ATTR amyloidosis
- 属性稳定器, a class of medications that may help stop TTR from forming amyloid fibrils in people with familial or wild-type ATTR amyloidosis
Your doctors may also recommend other treatments to help manage potential symptoms and complications of ATTR amyloidosis.
例如，这些支持治疗可能包括饮食变化，利尿剂或手术以帮助治疗心力衰竭。
在临床试验中还研究了其他针对ATT淀粉样变性的治疗方法，包括可能有助于清除体内淀粉样蛋白纤维的药物。
If you have ATTR amyloidosis, talk to your doctor to learn more about your treatment options and long-term outlook.
Early diagnosis and treatment may help slow the development of the disease, relieve symptoms, and improve your life expectancy.
Your doctor’s recommended treatment plan will depend on the specific type of the disorder that you have, as well as the organs that are affected.
新的治疗方法也可用傅ture to help improve survival rates and quality of life in people with this condition.
Your doctor can help you learn about the latest treatment developments.
