副肿瘤性天疱疮伴发Castleman＇s病和间质性肺炎

目的总结首例伴发Castleman＇s病和间质性肺炎的副肿瘤性天疱疮（PNP）的诊治经验。方法结合患者临床表现和组织病理、免疫病理和影像学检查结果诊断为PNP伴Castleman＇s病和间质性肺炎。用大剂量丙种球蛋白中和抗体,手术切除肿瘤,用大剂量激素冲击疗法治疗间质性肺炎。结果患者临床症状均缓解,随访至今无复发。结论PNP不但与内脏肿瘤相关,也可累及呼吸系统;手术切除、大剂量丙种球蛋白及激素冲击疗法是该病的有效治疗方法。     

Respiratory bronchiolitis associated with interstitial lung disease and desquamative interstitial pneumonia

This article explores issues of the diagnosis and management of respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia. These three diseases have common and overlapping features and sometimes are viewed as a continuum of smoking-induced disease, rather than as distinct and separate entities.     

Clinical evaluation of idiopathic interstitial pneumonia and interstitial pneumonia associated with collagen vascular disease using logistic regression analysis

OBJECTIVE: To clarify the differences in the clinical features between idiopathic interstitial pneumonia (IIP) and interstitial pneumonia associated with collagen vascular diseases (CVD-IP). METHODS: Symptoms, radiographic findings, pulmonary function, blood chemistry data including autoantibody, and bronchoalveolar lavage fluid (BALF) findings were compared using multiple logistic regression analysis. PATIENTS: The subjects were 44 patients clinically diagnosed with IIP and 33 patients with CVD-IP. RESULTS: The clinical features related to IIP were as follows: male sex, advanced age, past history of hypertension, presence of cough, exertional dyspnea, digital clubbing, an increased level of gamma-globulin, decreased lung volume on chest X-ray, and typical type according to the criteria for IIP on chest X-ray. Increased levels of rheumatoid factor and total cell number in BALF were related to CVD-IP. CONCLUSION: These findings are considered to be useful to differentiate IIP and CVD-IP.     

Lung cancer associated with idiopathic interstitial pneumonia

Idiopathic interstitial pneumonia (IIP) is known to have a tendency to be associated with lung cancer. Clinical characteristics of lung cancer associated with IIP are discussed in this article. Thirteen cases of lung cancer associated with IIP were examined from 1975 to 1988. A total of 590 cases of lung cancer and 38 cases of IIP were observed during that period. The 13 cases of lung cancer were found to be associated with IIP during the follow-up observation of our patients. Of these 13 patients, 12 men and a woman, the average age was 68.0 years old. All these 13 cases were ex- or present smokers. Five cases of lung cancer had no symptoms, but were detected by abnormal shadows on chest X-ray. Eight cases were detected symptoms. The duration from the onset of IIP to the onset of lung cancer was 36.5 +/- 23.5 months. Primary sites of lung cancer were distributed as follows. Nine cancers were in the left lung (64%) and five cancers were in the right lung (36%). Three cancers were in the hilar region (21%) and 11 cancers were in the peripheral lung field (79%). The numbers of lung cancers were equal in upper and lower lobe. The most common histological type was squamous cell carcinoma, followed by adenocarcinoma. Most cases were in stage III or IV. Three cases were operated, 4 were treated with chemotherapy, 4 were treated with chemotherapy plus irradiation, and 2 cases were treated by BRMs. The frequency of weight loss or finger clubbing in these patients was higher than in IIP patients without lung cancer.(ABSTRACT TRUNCATED AT 250 WORDS)     

Acute pulmonary thromboembolism associated with interstitial pneumonia

A 68-year-old woman, who had been diagnosed as idiopathic interstitial pneumonia, complained of progressive dyspnea on exertion for a week. Although her chest radiograph did not worsen, arterial blood gas findings were markedly worsened. Contrast-enhanced chest computed tomography showed filling defects of the right upper and middle lobe branches of the pulmonary artery. She was diagnosed as having acute pulmonary thromboembolism (APTE). Clinical symptoms and contrast-enhanced chest computed tomography findings were remarkably improved after the treatment with heparin and urokinase. APTE should be considered as a differential diagnosis in patients with interstitial pneumonia who have worsening of respiratory symptoms with unchanged chest radiograph.     

Usual interstitial pneumonia associated with psoriasis vulgaris]

The patient was a 56-year-old man who had received diagnoses of psoriasis vulgaris at the age of thirty-three and of nephrotic syndrome at forty-five, and had been treated with prednisolone. He consulted the outpatient clinic because of a cough, and interstitial pneumonia was diagnosed. He was admitted to our hospital because his symptoms and chest radiographic findings continued to worsen under treatment with antitussives. Chest radiography revealed a linear-reticular shadow in the lower lung fields. Chest computed tomography also revealed micro-cystic lesions, reticular shadows and traction bronchiectasis underneath the pleura at the back of both lower lobes. His serum titer of antinuclear antibody was increased, but he had no other symptoms or autoantibodies. Surgical lung biopsy under thoracoscopy revealed usual interstitial pneumonia (UIP). The association of interstitial pneumonia with psoriasis vulgaris is rare.     

结缔组织病相关性间质性肺炎与特发性间质性肺炎的鉴别

结缔组织病(connective tissue diseases,CTD)或称胶原血管病是一组以机体各个部位结缔组织成分损害为共同特征的自身免疫性疾病[1].许多结缔组织病如系统性红斑狼疮(SLE)、类风湿性关节炎(RA)、系统性硬化病(SSc)、多发性肌炎和皮肌炎(PM/DM)、干燥综合征、混合性CTD以及强直性脊柱炎等均可伴发间质性肺疾病,特别是间质性肺炎.     

Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease

BACKGROUND: Desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) are uncommon forms of interstitial lung disease and have been incompletely characterized. STUDY OBJECTIVES: To further characterize the clinical features and course of subjects with DIP and RB-ILD. DESIGN: Retrospective study. SETTING: Tertiary care, referral medical center. PATIENTS: Twenty-three subjects with DIP and 12 subjects with RB-ILD seen over a 12-year period between 1990 and 2001. INTERVENTIONS: None. RESULTS: The study population included 19 men (54%) and 16 women (46%). The mean (+/- SD) age at diagnosis was 46 +/- 10 and 43 +/- 7 years, respectively, for patients with DIP and RB-ILD. All subjects were either current or previous smokers except for three subjects with DIP. The diagnosis was confirmed in all cases by surgical lung biopsy. Bronchoscopy with transbronchial lung biopsy had been performed in 12 patients and was nondiagnostic in all. The most common pulmonary function abnormality was a reduced diffusing capacity of the lung for carbon monoxide. A CT scan of the chest revealed ground-glass opacities bilaterally in most patients who had DIP and RB-ILD. No differences were observed between subjects with DIP and RB-ILD with respect to clinical features, radiologic findings, or pulmonary function test results. The clinical course was characterized by relative stability in the majority of patients in both groups and a partial response to corticosteroid therapy. Five deaths were observed, including three resulting from progressive diffuse lung disease, all in subjects with DIP. CONCLUSIONS: We concluded that DIP and RB-ILD are chronic disease processes that in most patients are related to smoking. Persistent abnormalities can be seen on pulmonary function testing and radiologic studies despite smoking cessation and corticosteroid therapy. Corticosteroid therapy appeared to be associated with modest clinical benefit but usually not with resolution of disease. Progressive disease with eventual death can occur in subjects with DIP, especially with continued cigarette smoking.