The historical background to the disorder is unfolded. It was originally regarded as a dermatological curiosity, and later sarcoidosis was recognized as a disorder diffusely involving most tissues of the body. Clinical syndromes described include those presenting to the dermatologist, ophthalmologist, chest physician and radiologist, gastroenterologist and neurologist. Tissues commonly involved are lungs, lymph nodes, eyes, skin and bone in that order of frequency. Granulomatous uveitis in the presence of skin lesions should always arouse the suspicion of sarcoidosis; ocular and bone involvement are frequently associated with lupus pernio, whereas lymphadenopathy and splenomegaly are more commonly linked with plaques and maculo-papular eruptions. Bone cysts are rare in the absence of skin lesions, so routine radiography of hands and feet is of little diagnostic value. The basic criteria for establishing the diagnosis of sarcoidosis are twofold: (a) Suggestive clinical and/or radiological features with evidence of generalized involvement. (b) Histological proof of sarcoid tissue from at least one tissue. Evidence of one without the other is insufficient, for clinical or radiological manifestations alone present too wide a differential diagnostic problem and, conversely, isolated histological evidence of sarcoid tissue could be construed as a local sarcoid-tissue reaction. Histological confirmation is obtained whenever possible from accessible involved tissues (skin or lymph node). Otherwise blind biopsy of liver, scalene lymph node or gastrocnemius muscle is employed, or alternatively the Siltzbach-Kveim test is performed. It is a safe, simple and specific outpatient skin test, which provides histological confirmation in three-quarters of patients with sarcoidosis. The natural history of sarcoidosis and the response to treatment vary with the type of disease--namely subacute (transient) or chronic (persistent) forms of sarcoidosis. The only treatment which favourably influences clinical, or radiological or histological features of the disease is corticosteroid therapy. Indications for treatment are: ophthalmic involvement, steadily worsening chest radiograph, breathlessness, persistent hypercalciuria, disfiguring skin lesions, neurological involvement, disordered glandular function.
该疾病的历史背景得以展现。它最初被视为一种皮肤科的罕见病,后来结节病被认定为一种广泛累及身体大多数组织的疾病。所描述的临床综合征包括那些皮肤科医生、眼科医生、胸科医生、放射科医生、胃肠病学家和神经科医生所面对的情况。常见受累组织依次为肺、淋巴结、眼、皮肤和骨骼。存在皮肤病变时的肉芽肿性葡萄膜炎应始终引起对结节病的怀疑;眼部和骨骼受累常与冻疮样狼疮相关,而淋巴结病和脾肿大更常与斑块及斑丘疹相关。在无皮肤病变时骨囊肿罕见,因此手足的常规放射检查诊断价值不大。确立结节病诊断的基本标准有两条:(a) 具有提示性的临床和/或放射学特征并有全身受累的证据。(b) 至少一个组织有结节病组织的组织学证据。仅有其一而无另一条是不够的,因为仅临床或放射学表现存在过于广泛的鉴别诊断问题,相反,孤立的结节病组织的组织学证据可能被解释为局部结节病组织反应。只要有可能,就从易于获取的受累组织(皮肤或淋巴结)获得组织学证实。否则采用肝脏、斜角肌淋巴结或腓肠肌的盲目活检,或者进行Siltzbach - Kveim试验。这是一种安全、简单且特异的门诊皮肤试验,能在四分之三的结节病患者中提供组织学证实。结节病的自然病程及对治疗的反应因疾病类型而异,即亚急性(短暂性)或慢性(持续性)结节病形式。唯一能对疾病的临床、放射学或组织学特征产生有利影响的治疗是皮质类固醇疗法。治疗指征为:眼部受累、胸部X线片持续恶化、呼吸困难、持续性高钙尿症、毁容性皮肤病变、神经受累以及腺体功能紊乱。