英国医学课程作业

摘要-Abstract:

 

肉状瘤病是一种会导致炎症,原因不明的疾病。肉状瘤病会影响体内任何器官,但最常见的是影响你的肺、淋巴结、眼睛和皮肤。疾病的过程因人而异。

 

关键词:结节病、肺、淋巴结、肉芽肿性疾病

 

引言-Introduction

 

肉状瘤病是年代系统性肉芽肿性疾病,主要影响人体的肺和淋巴系统。这种疾病通常包括在肺中肉芽肿的形成。其他一般涉及的器官系统包括淋巴结、皮肤、心血管、眼睛,神经系统,肌肉骨骼、肾、内分泌系统。哈钦森在1877年首次描述肉状瘤的病例(1)。

 

病理生理学-Pathophysiology

 

肉状瘤病的病因尚不清楚。最近的报告显示高丙种球蛋白血症的细胞活动增加,迟发型过敏的降低,免疫失调都与肉状瘤病有关。遗传(HLA-B8 HLA-A1 HLA-DR3)以及环境因素也都是有关系的。常见的传染性病原体有结核分枝杆菌、支原体物种,棒状杆菌物种,螺旋体,非典型分枝杆菌,痤疮丙酸杆菌,伯氏疏螺旋体、单纯疱疹病毒、丙型肝炎病毒,巴尔病毒、巨细胞病毒、柯萨基病毒、风疹病毒、组织胞浆菌属物种,隐球菌物种,球孢子菌病,孢子丝菌病(2 - 3)。

 

肉状瘤病-The Sarcoidosis disease

 

摘要-Abstract:

 

Sarcoidosis is a disease of unknown cause that leads to inflammation. Sarcoidosis can affect virtually any organ, but most commonly it affects your lungs, lymph nodes, eyes and skin. The course of the disease varies from person to person.

 

Key Word: Sarcoidosis, Lungs, Lymph Node, Granulomatous disease

 

引言-Introduction

 

Sarcoidosis is s systemic granulomatous disease that primarily affects the lungs and lymphatic systems of the body. The disease most commonly involves granuloma formation in the lungs. Other commonly involved organ systems include the lymph nodes, dermatology, cardiovascular, opthalmology, nervous, musculoskeletal, renal, and endocrine systems. Hutchinson in 1877 first time described a case of sarcoid (1).

 

病理生理学-Pathophysiology

 

The etiology of sarcoidosis is unknown. Recent report have shown an increase in B-cell activity with hypergammaglobulinemia, reduced delayed-type hypersensitivity, immune dysregulation is responsible for sarcoidosis. Genetic (HLA-B8, HLA-A1, HLA-DR3) as well as environmental factors are also responsible. The common infectious agents implicated are Mycobacterium tuberculosis, Mycoplasma species, Corynebacteria species, spirochetes, atypical mycobacteria, Propionibacterium acnes, Borrelia burgdorferi, herpes simplex virus, hepatitis C virus, Epstein-Barr virus, cytomegalovirus, coxsackievirus, rubella virus, Histoplasma species, Cryptococcus species, coccidioidomycosis, and sporotrichosis (2-3).

 

临床特征-Clinical Feature

 

Symptoms can appear suddenly and then just as quickly resolve spontaneously. Symptoms can be related to the specific organ affected, or non-specific general symptoms including: weight loss , loss of apetite, fatigue, fever, chills and night sweats.

 

Cutaneous involvement is seen in 25% of patients and usually accompanies systemic involvement. It is either specific or nonspecific. Erythema nodosum (EN) is the main nonspecific cutaneous disease; lupus pernio, maculopapular, nodular, scar, plaque, angiolupoid, ichthyosiform, lichenoid, psoriasiform, and ulcerative lesions and subcutaneous nodules are examples of specific cutaneous disease. EN is usually an acute, self-limiting process and rarely requires treatment. Tender, erythematous nodules are usually present on the extremities, most commonly on the anterior surface of the tibia. Lupus pernio is characterized by red-to-purple or violaceous, indurated plaques and nodules that usually affect the nose, the cheeks, the ears, and the lips, but it can appear on the dorsa of the hands, the fingers, the toes, and the forehead. Plaque sarcoidosis is characterized by round-to-oval, red-brown to purple infiltrated plaques; the center of the plaque may be atrophic. They most commonly occur on the extremities, the face, the scalp, the back, and the buttocks, and they may have an annular appearance. Subcutaneous nodular sarcoidosis is also called Darier-Roussy sarcoidosis. : Lesions are usually nontender, firm, oval, flesh-colored or violaceous nodules that are 0.5-2 cm in diameter. They are commonly found on the extremities or the trunk(3-5).

 

Pulmonary system: Respiratory symptoms involved in more than 90% of patients presents with coughing, shortness of breath, chest tightness or chest pain, hemoptysis, Clubbing and crackles. Parenchymal lung disease (larynx, trachea, bronchi) may lead to airway obstruction and bronchiectasis, airway hyperactivity. In 5% pleural effusion (Lymphocytic), chylothorax, pneumothorax, pleural thickening, lymph node calcification and cavity formation can occur.

 

阶段成果-Stage Findings

 

0 Normal Chest X-ray

 

Bilateral hiliar lyphadenopathy (BHL)

BHLplus pulmonary infiltrations

Pulmonary infiltrations (without BHL)

Pulmonary fibrosis

Lymphatic system: palpable lymph nodes that are moveable and nontender. Right paratracheal lymph node enlargement is most commonly seen, followed next by cervical nodes.

 

Ocular involvement: Anterior uveitis is the most common finding, which may be associated with fever and parotid swelling (also called uveoparotid fever). Chronic uveitis, glaucoma, cataract and blindness. Lacrimal gland enlargement, keratoconjunctivitis sicca, dacrocystitis and retinal vasculitus.

 

Neurosarcoidosis: Granulomas can appear in the brain, spinal cord, and facial and optic nerves . May result in headache, confusion and malaise . Seventh cranial nerve palsy is the most frequent finding. The pituitary gland and the hypothalamus may be involved.

 

Myocardial involvement: Symptoms and signs associated with congestive heart failure, pericarditis or papillary muscle dysfunction. 24 hour Holter may reveal VT, heart block or V. ectopic beats and Doppler Echocardiogram showing diastolic dysfunction (3-4).

 

Other organs may be involved: 20% patients have hepatomegaly or changes in their liver enzyme levels, arthritis, proximal muscle weakness, anemia, leukopenia, hypercalcemia, diabetes insipidus, and renal failure may be noted.

 

Raised serum calcium levels in 2-63% of sarcoidosis patients due to overproduction of vitamin D or dysregulated production of 1.25-(OH)2-D3 (calcitrol)by sarcoid granulomas (5-7).

 

调查-Investigation

 

CBC count : Leukopenia and/or thrombocytopenia are frequent findings. Eosinophilia occurs in 24% of patients, and anemia occurs in 5% of patients. Hypercalciuria may be present. These levels may be elevated with hepatic and renal involvement. Elevated liver function tests suggestive of hepatic dysfunction, elevated erythrocyte sedimentation rate, elevated anti-nuclear antibodies (30%), diabetes insipidus, and renal failure may be noted.

 

Chest radiography is used in staging the disease. Stage I disease shows bilateral hilar lymphadenopathy (BHL). Stage II disease shows BHL plus pulmonary infiltrates. Stage III disease shows pulmonary infiltrates without BHL. Stage IV disease shows pulmonary fibrosis.

 

CT of the thorax may demonstrate lymphadenopathy or granulomatous infiltration.

 

Whole body gallium Ga 67 scanning : The lambda pattern is produced by uptake of the right paratracheal and bilateral hilar lymph nodes. The panda image is produced by symmetric uptake by the lacrimal and parotid glands.

 

Kveim test : involves intradermal injection of tissue from the spleen or the lymph node of a patient with sarcoidosis. A biopsy sample is obtained from the area 4-6 weeks after injection, and it is histologically examined for noncaseating granuloma formation, which, if found, indicates a positive result.

 

Tuberculin skin test : Patients with sarcoidosis have impaired delayed-type immune reactions.

 

Pulmonary function test : Common abnormalities found are defects in diffusing capacity and vital capacity.

 

Bronchoalveolar lavage with a CD4/CD8 ratio: A CD4/CD8 ratio of more than 3.5 has a specificity of 94% for sarcoidosis.

 

Biopsy of all cutaneous lesions of sarcoidosis, except EN, is helpful because, histologically, EN is not specific for sarcoidosis.

 

Histopathology: Typical sarcoid lesions are characterized by the presence of circumscribed granulomas of epithelioid cells with little or no necrosis . Granulomas are usually in the superficial dermis, but they may involve the thickness of the dermis and extend to the subcutaneous tissue. Islands of epithelioid cells may contain a few Langhans giant cells. Giant cells may contain asteroid or Schaumann bodies. Asteroid bodies are star-shaped eosinophilic structures. Schaumann bodies are round or oval, laminated structures, which are usually calcified at the periphery (3-5).#p#分页标题#e#

 

治疗-Treatment

 

Oral corticosteroids are usually the treatment of choice for patients with neurologic, cardiac, or ocular involvement not responding to topical corticosteroids; hypercalcemia; and symptomatic stage II and all stage III pulmonary disease. The usual dose is 30-40 mg of prednisone daily for 2-3 months, with a gradual taper over 1 year to 10-20 mg every other day. Patients with severe disease may need prednisone doses up to 1 mg/kg/d. Symptomatic relief can be obtained using nonsteroidal anti-inflammatory drugs (NSAIDs).

 

Limited, nondisfiguring cutaneous involvement may be treated with topical or intralesional corticosteroids. Intralesional injections of 2-10 mg/mL of triamcinolone acetonide can be used at monthly intervals. More chronic skin lesions, such as plaques or lupus pernio, require more aggressive therapy because theycan lead to permanent scarring. If intralesional corticosteroids are not effective,other standard therapies includesystemic corticosteroids, methotrexate, and ]antimalarials (hydroxychloroquine and chloroquine) can be used.

 

Tumor Necrosis Factor Blockers: TNF enhances alveolar macrophages activity in active sarcoid, and will lead to increase granuloma formation, and disease activity. TNF secretion ( or action) can be decreased by the use of various drugs such as corticosteroids, MTX, Pentoxyfylline, Thallidomide and Infliximab

 

Radiation has also been used to treat treatment-resistant cutaneous lesions (1,3-4).

 

Surgical excision of small lesions or excision of larger lesions with skin grafting can be attempted. Laser surgery using carbon dioxide and pulsed dye laser has also been used.

 

引用-References:

 

Baumann RJ, Robertson WC Jr: Neurosarcoid presents differently in children than in adults. Pediatrics 2003;112:e480-e486.

Hoffman AL, Milman N, Byg K-E: Childhood sarcoidosis in Denmark 1979-1994: Incidence, clinical features and laboratory results at presentation in 48 children. Acta Paediatr 2004;93:30-36.

Shetty AK, Gedalia A: Sarcoidosis in children. Curr ProbI Pediatr 2000;30:149-176.

Lannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med 2007;357:2153-65.

Belfer MH, Stevens RW.Sarcoidosis: a primary care review.Am Fam Physician.Dec1998;58(9):2041-50, 2055-6.

du Bois RM.Corticosteroids in sarcoidosis: friend or foe?.Eur Respir J.Jul1994;7(7):1203-9.

Young C, Burrows R, Katz J, Beynon H.Hypercalcaemia in sarcoidosis.Lancet.Jan 301999;353(9150):374.