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结节病

Overview

结节病 is a disorder that causes inflamed tissue, called nodules or granulomas, to develop in the body's organs, 最常见的是肺. 它也会影响皮肤, 眼睛, 鼻子, 肌肉, 心, 肝, 脾, 肠, 肾脏, 睾丸, 神经, 淋巴结和大脑. Nodules in the lungs can lead to narrowing of the airways and inflammation, 也称为纤维化, 肺组织.

结节病 affects people of all ages, 种族和性别, though it most commonly occurs in people between 20 to 40 years old. Children are rarely diag鼻子d with the disease. In very few cases, more than one family member is affected. African-Americans are three to four times more likely to have sarcoidosis and may have a more severe form of the disease than people of European descent.

Our approach to sarcoidosis

Many cases of sarcoidosis resolve on their own and cause no lasting problems. 如果情况持续, UCSF offers specialized care that includes 药物治疗s to relieve 症状 and decrease inflammation. Our specialists review each case as a team to ensure that every patient gets the right diagnosis and most effective care.

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迹象 & 症状

Symptoms of sarcoidosis may vary from person to person, and depend on the organs affected. Frequently, the condition causes mild 症状 and resolves on its own without treatment. In approximately half of all patients, sarcoidosis is detected on a routine 胸部x光片 before any 症状 develop. The most common 症状 of sarcoidosis involving the lungs include:

  • 咳嗽
  • 呼吸急促(气促)
  • 胸部疼痛, which is usually a vague tightness of the chest, but can occasionally be severe and similar to the pain of a 心 attack
  • 乏力
  • 弱点
  • 发热
  • 减肥

诊断

In making a diagnosis of sarcoidosis, your doctor will begin by conducting a thorough physical examination, asking about any 症状 you are experiencing, including when they started and how they've progressed or eased over time. Your full medical history will also be recorded.

There is no singular finding that confirms a diagnosis of sarcoidosis. Therefore, the diagnosis is based on multiple factors, including 症状, abnormalities on a 胸部x光片 or CT扫描, and microscopic examination of one or more specimens from involved tissues or organs.

除了, you may also have the following 测试s to help rule out other conditions, 比如肺结核, which cause similar 症状 as sarcoidosis, and to make a definite diagnosis.

  • Pulmonary function 测试ing (PFT). 这 测试 involves a series of breathing maneuvers that measure the airflow and volume of air in your lungs. 这 allows your doctor to objectively assess the function of your lungs. PFT is recommended for all patients.
  • High resolution computed tomography (HRCT). 这是一种特殊的 CT扫描 that provides your doctor with high-resolution images of your lungs that are extremely valuable in determining which type of Interstitial Lung Disease you have. Having a HRCT is no different than having a regular CT扫描; they both are performed on an open-air table and take only a few minutes. HRCT is recommended for all patients.
  • 六分钟步行测验. 这 测试 is a formal evaluation of the distance you can walk and the oxygen saturations (measured by finger or ear probe) you achieve while walking. 这 is an important marker of functional status used by your doctor in managing your condition. The six minute walking 测试 is recommended for all patients.
  • 支气管镜检查.测试 involves the passage of a flexible fiberoptic scope about the diameter of a pencil into the lungs to obtain fluid and tissue samples to aid in diagnosis. 这 测试 is an outpatient procedure, which means you do not have to stay overnight in the hospital, and is performed by your doctor. 支气管镜检查 is recommended for some patients.

Once your diagnosis is made, additional 测试s may be needed to determine the stage and severity of your disease. These may include 测试s to check for sarcoidosis involving other organs.

治疗

结节病 is treated at the UCSF Interstitial Lung Disease (ILD) Program. World-renowned lung specialists, chest radiologists and pathologists work together as a team to design the most effective treatment plan for your individual condition.

The cause of sarcoidosis is unknown at this time. Therefore, there is no specific treatment to cure the condition. 幸运的是, 在很多情况下, sarcoidosis does not require treatment because the nodules gradually resolve on their own and leave behind few, 如果有任何, signs of inflammation or other complications.

However, treatment is necessary in some cases. Medications are available that effectively suppress 症状 and help reduce lung inflammation, the impact of nodules and prevent the development of lung fibrosis. These include the following 药物治疗s.

糖皮质激素

糖皮质激素,通常 强的松, are particularly effective in reducing inflammation, and are typically the first drugs used in the treatment of sarcoidosis. In patients with mild 症状, 比如皮肤损伤, 眼睛发炎, 或咳嗽, topical steroid therapy with creams, eye-drops or inhalers may be sufficient to control the disease. When necessary, oral steroids are generally prescribed for six to twelve months. 在大多数情况下, a relatively high dose is prescribed at first, followed by a slow taper to the lowest effective dose.

Symptoms, especially cough and shortness of breath, generally improve with steroid therapy. Relapses may occur after treatment with steroids has ended, but typically respond to repeated steroid treatment. 病人 who improve and remain stable for more than year after the end of treatment have a low rate of relapse.

Researchers continue to examine the role of steroids in the treatment of sarcoidosis, with some addressing the question of what effect they may have on the long-term course of the disease. However, in general, steroid therapy remains the leading treatment for sarcoidosis.

其他药物

Alternative 药物治疗s are used in patients who cannot tolerate steroids, do not respond to steroids or wish to lower the dose of steroids. 这些包括:

  • 甲氨蝶呤.药物治疗 reduces inflammation and suppresses the immune system.
  • 环磷酰胺.药物治疗 is commonly used in conjunction with steroids in patients whose condition is worsening despite treatment.
  • Antimalarial 药物治疗s (hydroxychloroquine). These 药物治疗s are used to treat sarcoidosis of the skin and lungs.
  • 秋水仙碱. 这 药物治疗 is most commonly used to treat gout and is sometimes prescribed to treat sarcoidosis-related arthritis.

A number of other 药物治疗s are currently being investigated for the treatment of sarcoidosis.

加州大学旧金山分校健康 medical specialists have reviewed this information. It is for educational purposes only and is not intended to replace the advice of your doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your provider.

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