肺曲菌球的外科治疗（附182例报告）

目的探讨肺曲菌球的手术适应证及手术治疗效果。方法回顾性分析我院1975年9月至2008年1月经手术治疗的182例肺曲菌球患者资料,分为单纯性肺曲菌球组（SPA,n：42）和复合性肺曲菌球组（CPA,n;140）。行肺叶切除术146例,全肺切除13例,复合切除（肺叶＋肺段或楔形切除）11例,肺段切除7例,楔形切除5例。结果182例患者中治愈178例,治愈率97．8％。无手术死亡,术后发生并发症47例（25．8％）,包括出血5例,持续漏气10例,肺炎11例,肺复张不全8例,支气管胸膜瘘、脓胸3例,心律失常3例,肺脓肿5例,切口感染2例。SPA组术后并发症发生率低于CPA组（P〈0．05）。术后随访173例,随访4个月～5年无复发。结论外科手术为肺曲菌球的首选治疗方法。对于病程短、肺病变局限、胸膜粘连较轻的患者电视胸腔镜具有创伤小、恢复快、术后并发症少的优点是其最佳选择。     

Results of surgical treatment for pulmonary aspergilloma with 26 cases in six years: a single center experience

Surgery for pulmonary aspergilloma is reputed to be risky. We analyzed our results of the surgical treatment for pulmonary aspergilloma. Between 2003 and 2009, 26 patients underwent thoracotomy for treatment of pulmonary aspergilloma in our center. Results were evaluated retrospectively. There were 5 female and 21 male patients, with a mean age of 44 ± 11.6 years (28-70). The patients were divided into two groups, group A (simple aspergilloma; n= 8) and group B (complex aspergilloma; n= 18). Major underlying diseases were tuberculosis (61.5%). The most common indication for operation was hemoptysis (57.6%). Of our patients, 23% were complaining of massive hemoptysis or recurrent hemoptysis. Other patients were complaining of mild symptoms and some of them were totally asymptomatic. We performed 15 (57.6%) lobectomies (3 with associated segmentectomies), 8 (30.6%) segmentectomies/wedge resections, 2 (7.6%) pneumonectomies, and 1 (3.8%) cavernoplasty. Postoperative complications occurred in 15 (57.6%) patients. Complications occurred in 72.2% patients of complex aspergilloma, whereas 25% occurred in simple aspergilloma (p= 0.03). Major complications included prolonged air leak, empyema, air space. One patient who underwent lobectomies for complex aspergilloma developed bronchopleural fistula and died of respiratory failure on the 20th postoperative day. Operative mortality was 3.8%. The average postoperative hospital stay was 12.9 days. The mean follow-up period was average 44 months. The actuarial survival at 3 years was 90% and 100% for complex aspergilloma and simple aspergilloma, respectively (p> 0.05). There was two recurrence of disease (8%). But no recurrence of hemoptysis. Low morbidity rate may have been due to the selection of patients with localized pulmonary disease in this study. Surgical resection of asymptomatic or symptomatic pulmonary aspergilloma is effective in preventing recurrence or massive hemoptysis for patients whose condition is fit for pulmonary resection with reasonable mortality, morbidity and survival rates.     

Analysis of surgical treatment for pulmonary aspergilloma

Surgery for pulmonary aspergilloma is reputed to be risky. The results of surgical treatment of pulmonary aspergilloma in 41 patients between 1988 and 2003 were evaluated retrospectively. Hemoptysis occurred in 31 patients (75.6%) and it was massive (> 300 mL in 24 hr) in 3. The underlying lung disease was tuberculosis in 35, bullous lung disease in 2, hydatid cyst in 2, and lung carcinoma in 2 patients. Lobectomy, bilobectomy, wedge resection, and pneumonectomy were performed in 27, 4, 6, and 4 patients respectively. The postoperative complication rate was 24.4%. One patient, who had a right pneumonectomy, died due to respiratory failure; the mortality rate was 2.4%. Recurrent hemoptysis was observed in only one patient. Early surgical treatment of patients with pulmonary aspergilloma resulted in a satisfactory outcome with acceptable morbidity, low mortality, and effective prevention of recurrent hemoptysis. Pneumonectomy has a high morbidity, thus it should be avoided if possible.     

Pulmonary aspergilloma--radiological observations

The radiological appearances of 49 cases of aspergilloma seen over a period of 6 years among 36,340 hospital admissions are described. All the 49 patients had pulmonary tuberculosis as underlying disease with 6 (12.2%) having bacteriologically active disease. One patient had concomitant allergic bronchopulmonary aspergillosis (ABPA). Upper zone distribution, large cavity size, moderately thick cavity wall and overlying pleural thickening were some of the prominent features observed. Two cases of multiple (3 each) and 4 of bilateral aspergilloma were seen. Of 57 aspergillomas 47 were round or oval, 7 oblong, 2 polypoidal and 1 lobulated. Positional movement was observed in 30 cases. Spontaneous lysis was seen in one case. Tomography and lordotic view were found to be very useful techniques when postero-anterior films were unrevealing. The radiologic diagnosis of aspergilloma was confirmed by demonstration of serum precipitins against aspergilli in 44 cases.     

Intracavitary amphotericin B in the treatment of symptomatic pulmonary aspergillomas

The optimal treatment of pulmonary aspergillomas is not established. Surgical resection is often impossible because of severe, underlying pulmonary impairment, and medical treatment has given negative or inconclusive results. Six patients with symptomatic pulmonary aspergillomas were treated with percutaneous instillation of intracavitary amphotericin B. Four patients who received the full course of therapy showed improvement and stabilization or reversal of progressive roentgenographic changes. Also, follow-up serologic studies of Aspergillus spp. precipitins were obtained in three and were negative. One patient did not tolerate this treatment because of repeated systemic reactions. Another patient did not respond clinically or roentgenographically. Intracavitary amphotericin B therapy should be considered in patients with symptomatic pulmonary aspergilloma, particularly when surgical resection is not feasible.     

Ventricular septal defect with increased pulmonary vascular resistance. Late results of surgical closure

The fate of 57 children who underwent surgical closure of ventricular septal defect in the presence of increased pulmonary vascular resistance was analyzed. Eighteen (32 percent) died at or immediately after operation. Seven late deaths, due to the Eisenmenger syndrome, occurred 1 to 7 years after operation. Follow-up studies, including cardiac catheterization, were performed in 25 survivors, 1 to 11 years (mean 5 years) after operation. In patients operated on after age 2 years, the degree of preoperative elevation of pulmonary vascular resistance largely determined the prognosis: The condition of patients with mild elevation (pulmonary vascular resistance less than one third the systemic level) returned to normal after operation, with one exception. Patients with more severe elevation (pulmonary vascular resistance more than one third the systemic level) most often showed progressive pulmonary vascular disease; the condition of a few remained unchanged and that of only one returned to normal after operation.Operative mortality rate was high in children with elevated pulmonary vascular resistance during the second year of life who were operated on before age 2 years, but pulmonary vascular resistance returned to normal in the three survivors. When pulmonary vascular resistance was increased in the first year of life, it generally decreased spontaneously (independently of operation); the early increase in these cases is believed to represent delayed maturation of the pulmonary vascular bed rather than pulmonary vascular disease.The overall results of surgery in ventricular septal defect associated with a high level of pulmonary vascular resistance are unfavorable and call for interruption of the natural history before significant pulmonary vascular obstructive changes have taken place; this can be achieved by surgical closure before age 2 years or pulmonary artery banding in infancy.     

Prognosis of patients with primary pulmonary hypertension

Primary pulmonary hypertension is a rare disease entity. Clinical evaluations of such patients were deemed inadequate during a multi-center study in Japan (1976). The prognoses of our patients in Keio Hospital were evaluated, especially in terms of their clinical pictures and hemodynamic backgrounds. The study group consisted of 15 patients, who fulfilled the clinical criteria of primary pulmonary hypertension according to the Research Committee of the Ministry of Health and Welfare in Japan. There were four males and 11 females, whose age ranged from 16 to 72 years and averaged 34.7 years. Follow-up periods from the onsets were from 15 to 152 months, and they averaged 51 months. Nine of the 15 patients were deceased, and autopsies were performed in eight. The periods from onsets to deaths were between 15 and 152 months, and averaged 53 months (4 years and 5 months). Except for four cases followed for less than 2 years, there were seven who survived over three years and four patients who were deceased in this period, but there were no differences between these groups in terms of their clinical pictures, such as age, sex and symptomatology at onset. Survivors included three patients associated with thyroid disease, one with liver cirrhosis, and one with the Sj?gren syndrome. Hemodynamically, heart rates and pulmonary artery pressures did not differ among six survivors more than 2 years after the hemodynamic evaluations and five patients who were deceased within this period. In the deceased group, however, cardiac output was low and arterio-venous differences in oxygen content were high.(ABSTRACT TRUNCATED AT 250 WORDS)     

Deaths in risk classes I-III: a measure of quality of care in patients hospitalised with CAP?

All deaths occurring in patients with community-acquired pneumonia in risk classes I-III were reviewed as a quality-of-care measure. The immediate and underlying causes of death were classified according to the World Health Organization protocol. Eleven (1.8%) of the 608 low-risk patients died. Three of the patients did not have pneumonia, one of whom was admitted with atypical pulmonary oedema due to stenosis of a prosthetic heart valve. Failure to include chronic lung disease in the severity-of-illness scoring system resulted in misclassification of seven patients. The most common underlying causes of death were pulmonary fibrosis at 27%, chronic obstructive lung disease at 18% and cancer at 27%. Respiratory failure was the immediate cause of death in 64% of patients, cardiac causes in 27%, and pneumonia in 9%. To conclude, the review of deaths in patients at low risk for mortality is useful for monitoring the quality of care received by patients who require admission for the treatment of community-acquired pneumonia, and that the pneumonia-specific severity-of-illness scoring system results in misclassification of patients with chronic obstructive lung disease and pulmonary fibrosis.     

呼吸系统曲霉菌病重叠综合症的新进展

肺部曲霉菌病是少见病,但近年逐年增多,包括变应性支气管肺曲菌病(allergic bronchopulmonary aspergillosis,ABPA)、曲菌球(aspergilloma)、慢性坏死性肺曲菌病(chronic necrotizing aspergillosis,CNA)、侵袭性肺曲菌病(invasive pulmonary aspergillosis,IPA)及全身播散性曲菌病等5型.其中ABPA、曲菌球甚至IPA可合并变应性曲霉菌性鼻窦炎(allergic Aspergillus sinusitis,AAS),可能与基础病及其治疗等因素有关.本文就近期文献报告的呼吸系统曲霉菌病重叠综合症作一综述.     

The causes of death of pulmonary tuberculosis: late sequelae of pulmonary tuberculosis

We investigated the causes of death of late sequelae of pulmonary tuberculosis. Chronic respiratory failure is one of the most frequent cause of death in the patients of late sequelae of pulmonary tuberculosis. We compared the long term prognosis of chronic respiratory failure in case of emphysema and pulmonary tuberculosis. In the patients with chronic respiratory failure by pulmonary emphysema, the prognosis was poor in those with pulmonary hypertension. But in case of late sequelae of pulmonary tuberculosis, prognosis was not affected by presence or absence of pulmonary hypertension. The determinants of prognosis of late sequelae of pulmonary tuberculosis are the indication of home oxygen therapy, malnutrition, and hypoxemia. Fungal infection, especially aspergilloma, is a common secondary infection of late sequelae of pulmonary tuberculosis. We investigated forty-two cases of aspergilloma as late sequelae of pulmonary tuberculosis, and of those 15 patients died. The causes of death were pneumonia and respiratory failure. Measurement of galactomannan antigen of aspergillus in serum using ELISA or PCR, it was apparent that the outcome was poor in the patients positive for antigen. It suggested that the prognosis of the patients with aspergilloma related with some degree of invasion of Aspergillus in parenchyma. It was reported that neoplasm is closely related to chronic tuberculous empyema. Lymphoma is most frequently complicated with chronic tuberculous empyema, and squamous cell carcinoma, adenocarcinoma, sarcoma and carcinoid were reported as complication of chronic empyema. We reported the case of angiosarcoma, originated from chronic empyema in left thoracic cavity formed after being treated for tuberculosis with artificial pneumothorax. Recently, the number of patients with late sequelae of pulmonary tuberculosis have been decreased, but some severe cases of patients of pulmonary tuberculosis will suffer from late sequelae of pulmonary tuberculosis, and that is still a great problem of the clinical course of pulmonary tuberculosis.     

Initial experience with heart and lung transplantation

Between February 1983 and July 1987, twelve patients underwent heart-lung transplantation at the University of Cape Town and the University of Munich. The patients included eight men and four women, whose ages ranged from 15 to 49 years (mean, 27 years). The underlying pathologic condition was idiopathic primary pulmonary hypertension in five cases, Eisenmenger's syndrome in four cases, idiopathic pulmonary fibrosis in one case, diffuse fibrosing alveolitis in one case, and chronic emphysema in one case. The immunosuppressive regimen consisted of cyclosporine A, azathioprine, and rabbit antithymocyte globulin (RATG) during the first 2 postoperative weeks; RATG was subsequently replaced by methylprednisolone. Pulmonary rejection frequently occurred in the absence of cardiac rejection; in one case, however, this situation was reversed. Two patients required retransplantation, which was undertaken for caseating pulmonary tuberculosis with obliterative bronchiolitis after 1 year in one case and for early pulmonary insufficiency after 2 days in the other case. There were no operative deaths, but three early deaths occurred, owing to respiratory insufficiency of unknown origin (10 days postoperatively), multiorgan failure (10 days postoperatively), and acute liver dystrophy (11 days postoperatively). Five weeks after operation, a fourth patient died of multi-organ failure. There were five late deaths, all of which resulted from infectious complications. Three patients, including one who underwent retransplantation, remain alive and well, 10 to 36 months postoperatively.     

In-hospital mortality in the Pickwickian syndrome

Ten patients were observed during 11 episodes of progressive ventilatory failure secondary to gross obesity in a 5 year interval in one hospital. In-hospital mortality was high; seven patients died. Three deaths were related to hypoventilation. Despite effective therapy of ventilatory failure with improvement in clinical status and blood gases, four other patients died, three of pulmonary embolism and one of acute renal failure. In addition, in one surviving patient transitory renal failure developed. In treating patients with the Pickwickian syndrome, close surveillance, prompt recourse to supported ventilation and prophylactic anticoagulation are mandatory. Unexpected death may occur despite clinical and physiologic improvement.     

Place of surgery in pulmonary aspergillosis and other pulmonary mycotic infections

Surgery is part of the therapeutic strategy of aspergillosis and mucormycosis. The aspergilloma is defined as a rounded mass, developing in a cavity by the proliferation of spores of Aspergillus. The most common complication was haemoptysis reported in 50-95% of cases. The pleuropulmonary lesions predisposing are: tuberculosis, residual pleural space, emphysema and lung destroyed by fibrosis or radiotherapy or bronchiectasis. The indications for surgery depend on symptoms, respiratory function, the parenchyma and the type of aspergilloma (simple or complex). In a patient with an intrapulmonary aspergilloma, lung resection preceded by embolization is recommended based on respiratory function. For intrapleural aspergilloma, thoracoplasty is recommended according to the patient's general condition. The invasive pulmonary aspergillosis (IPA) is characterized by an invasion of lung tissue and blood vessels by hyphae in immunocompromised patients. The death rate of patients who have an API after treatment for leukemia or lymphoma was 30 to 40%, after bone marrow transplantation 60%, after solid organ transplantation from 50 to 60% and after any other cause of immunocompromising from 70 to 85%. The main cause of these deaths is massive hemoptysis. Surgery (lobectomy) is indicated for the prevention of hemoptysis when the mass is in contact with the pulmonary artery or one of its branches, and if it increases in size with the disappearance of border security between the mass and the vessel wall. The patient will be operated in an emergency before the white blood cells do not exceed the threshold of 1000 cells/μl. A persistent residual mass after antifungal treatment may justify a lung resection (lobectomy or wedge) before a new aggressive therapy. Mucormycosis affects patients following immunocompromising states--haematologic malignancy, diabetes mellitus, transplantation, burns and malnutrition. The treatment of pulmonary mucormycosis combines surgical and medical approach.     

肺曲菌球病63例临床分析

目的探讨肺曲菌球病的临床特征和治疗方案。方法回顾性分析2008.1~2014.10年收治63例病理确诊肺曲菌球病患者的临床诊治情况。结果 HCT37%24例,血清前白蛋白(PA)200 mg/L20例,LY1.5×10~9/L 19例;肺结核空洞22例,支气管扩张症19例,6例无明确基础疾病;咳嗽46例,咯血59例,大咯血29例。典型影像学表现48例,误诊4例,漏诊11例;可见纵隔淋巴结23例,短径小于1 cm,淋巴结活检提示反应性增生。病灶位于左肺上叶17例,右肺上叶29例。肺功能检查:总弥散量低于比弥散量(t=-9.422,P0.001);均行外科手术治疗;58例随访影像学未见复发;大咯血与相关危险因素的Logistic分析,肺结核空洞OR=6.113,P=0.008,空洞或空腔内长径OR=2.410,P=0.004。结论肺曲菌球病咯血和咳嗽为主要症状;病灶多位于双肺上叶,影像学特征典型,大咯血患者的曲菌球长径、空洞或空腔长内径长于非大咯血患者;弥散功能有一定特征性;首选手术治疗;肺结核空洞和空洞或空腔内长径是大咯血的危险因素。     

Invasive pulmonary aspergillosis

BACKGROUND: Invasive pulmonary aspergillosis usually occurs in immunocompromised patients. Mild abnormality of host defence is usually present in the chronic necrotising form of the disease. Acute aspergillus pneumonia usually affects patients who are seriously immunocompromised. OBJECTIVES: The purpose of the study was to highlight the possibility of occurrence of invasive pulmonary aspergillosis also in patients with mild abnormality of host defence. METHODS: In a retrospective study 6 patients were analysed. The inclusion criterion was evidence of Aspergillus sp. invasion in lung tissue. Lung tissue was obtained by biopsy or post mortem examination. RESULTS: There were 4 patients with acute aspergillus pneumonia. Two of them were severely immunocompromised - one with dermatomyositis, who was treated with high doses of corticosteroids and methotrexate, and the other with undiscovered miliary tuberculosis, who was treated for myelodysplastic syndrome instead with low doses of corticosteroids. The other 2 had mild immunosuppression: one was suffering from sarcoidosis and was treated with low doses of corticosteroids, the other had dilated cardiomyopathy, renal insufficiency and diabetes mellitus. The two patients with chronic necrotising pulmonary aspergillosis had mild abnormality of host defence: one had reactivation of tuberculosis and diabetes mellitus, the other had inactive tuberculosis and aspergilloma. CONCLUSIONS: Invasive pulmonary aspergillosis must be considered also in patients with mild immunosuppression and pulmonary infiltrates which do not respond to conventional treatment with antibiotic chemotherapy. The key to the diagnosis of invasive pulmonary aspergillosis is the histopathological demonstration of fungal invasion in lung tissue.     

Experience with the use of continuous positive airway pressure (CPAP) therapy in the emergency management of acute severe cardiogenic pulmonary oedema

Background: Acute pulmonary oedema (APO) is a frequent cause of respiratory failure and a common reason for presentation to emergency departments (EDs). To date, no paper has been published on the application of continuous positive airway pressure (CPAP) therapy for a large broad-based patient group.
Aim: To report our experience with the use of CPAP in severe APO oedema, with particular reference to safety, intubation rates and impact on EDs' resources.
Method: A retrospective chart review was undertaken of 75 patients with acute severe pulmonary oedema who were treated with adjuvant CPAP in an urban teaching hospital ED.
Results: Three patients (4%) required subsequent endotracheal intubation and mechanical ventilation. The average duration of CPAP was 1.9 hours. Eighty nine per cent of patients experienced no adverse events while being treated with CPAP. Five patients failed to tolerate the tight fitting mask necessitating removal of CPAP, three patients experienced arrhythmias related to underlying cardiac disease and two patients experienced mild transient hypotension. Seventy one per cent of patients were discharged from the ED to general medical wards. The in-hospital mortality for patients treated with CPAP was 15%.
Conclusion: This series has demonstrated that CPAP therapy delivered via a face mask for the treatment of acute severe APO is safe and effective when applied to a broad range of patients. We recommend the use of CPAP therapy for all suitable patients presenting in severe APO irrespective of age or underlying pulmonary disease.     

Clinical significance of scintigraphy in pulmonary aspergilloma]

In order to study the scintigraphic findings related to the pathophysiology of pulmonary aspergilloma, chest scintigraphy with T1-201 chloride and perfusion lung scintigraphy were performed in 18 and 19 patients with pulmonary aspergilloma, respectively. In T1-201 scintigraphy, accumulation of T1-201 in the diseased areas related to Aspergillus infection was observed in 18 of 19 foci. Roentgenographic findings related to the accumulation of T1-201 in the lesion were thickness of the cavity wall and pleural thickening adjacent to the cavity, but they showed no relation to the size of the fungus ball, and 2 cases without fungus ball showed positive results. Moreover, in T1-201 single photon emission computed tomography, accumulation of T1-201 was demonstrated in the area surrounding fungus ball. In addition, findings of inflammation such as leukocytosis, elevated erythrocyte sedimentation rate, positive CRP and fever were observed in more than 50% of all cases. These results suggest that tissues surrounding the fungus ball, including the cavity wall and thickened pleura undergo inflammatory process secondary to infection by Aspergillus. Perfusion lung scintigrams showed decreased perfusion in all of 19 cases, which was more marked than that expected from the chest radiograph in many cases. In T1-201 myocardial scintigraphy, visualization of the right ventricle was observed in 15 of 18 cases. T1-201 chest scintigraphy and perfusion lung scintigraphy are useful for evaluation of the activity of the disease process, right ventricular overloading, and regional perfusion in pulmonary aspergilloma.     

Short- and long-term experience with flecainide acetate in the management of refractory life-threatening ventricular arrhythmias

Thirty-eight patients with organic heart disease and history of sudden cardiac arrest or recurrent sustained ventricular tachycardia were treated with flecainide. Coronary artery disease was present in 33 patients. Previous antiarrhythmic therapy consisted of two to eight drugs (mean four). Fourteen patients were resuscitated from sudden cardiac death and 24 patients had chronic recurrent sustained ventricular tachycardia. Twenty-eight patients had electrophysiologic testing before and during flecainide treatment. Sustained ventricular tachycardia became noninducible in 5 patients, nonsustained in 5 patients and slowed in 13 patients (cycle length increased from 278 +/- 64 to 395 +/- 91 ms; p = 0.002). Three of the 14 patients with sudden cardiac death and 15 of the 24 patients with recurrent sustained ventricular tachycardia remained on long-term flecainide treatment. The mean left ventricular ejection fraction in 16 of these 18 patients was 37%. Nonlimiting side effects occurred in seven patients (18%). Proarrhythmic effects were seen in four patients (10%). At a mean follow-up time of 11 +/- 3 months, 15 patients (39%) had had no recurrence, including 5 who had inducible sustained ventricular tachycardia and 5 who did not on retesting during treatment. In the 18 patients who received long-term therapy, 3 late deaths occurred, 1 of which was of arrhythmic origin. These data suggest that flecainide is effective in about 40% of patients with severe refractory ventricular arrhythmias. Its value as a single drug in the treatment of sudden cardiac death remains to be defined.     

Single institutional experience with non-seminomatous germ cell tumours of the testis: local perspectives on a curable cancer

Abstract We have reviewed 77 patients with Non-seminomatous germ cell tumour of the testis (NSGCTT) treated at a single institution. A residual mass following definitive treatment occurred in 16 patients (35%), 13 of whom had a resection of the mass, yielding active tumour in only one patient. Nine patients (12%) relapsed including four of the 14 with Stage I disease who were treated by orchidectomy alone. Four relapses occurred at more than two and a half years after primary treatment. Relapse prior to the development of clinical symptoms or signs was evident in three of nine patients; in two patients by routine imaging and one with elevated routine serum markers. Three of the nine patients who relapsed had elevated serum markers. Two patients died from disease but there were four treatment-related deaths (7%). Overall, 64 patients (83%) remain disease free at the time of follow-up. A further seven (9%) have been lost to follow-up but were disease-free at a minimum of 26 months after diagnosis. This study confirms features of this disease including the excellent prognosis when adequately treated. However, it also reveals the problems of late or marker negative relapses, the implementation of an observation policy in Stage I disease and treatment related mortality in young men. (Aust NZ J Med 1991; 21: 857–862.)     

Severe pulmonary hypertension associated with macronodular (postnecrotic) cirrhosis and autoimmune phenomena

The clinical and pathologic findings are reported in seven consecutive patients with progressive and fatal pulmonary hypertension which was not explained by predisposing cardiac or pulmonary diseases. Pulmonary arterial lesions consisted of atherosclerosis of the elastic pulmonary arteries, and medial hypertrophy and concentric laminar fibrosis of the muscular pulmonary arteries in seven patients, plexiform lesions in six patients and necrotizing vasculitis in one patient. Pulmonary emboli were not identified. Five patients had manifestations of autoimmune disease, including laboratory abnormalities (positive antinuclear antibody, positive latex agglutination for rheumatoid factor, hypergammaglobulinemia or antimitochondrial antibody) in four, necrotizing vasculitis in one, Raynaud's phenomenon in two and clinical evidence of multisystem collagen vascular disease in two. Five patients had liver disease which developed prior to or concomitant with the onset of pulmonary hypertension. At autopsy, one patient had prominent periportal fibrosis and four had macronodular (postnecrotic) cirrhosis (active in three and inactive in one). Four of these five patients with liver disease and pulmonary hypertension had evidence of autoimmune phenomena. The findings in the seven patients suggest an association between autoimmune disease, plexogenic pulmonary hypertension and liver disease of the chronic active hepatitis-postnecrotic cirrhosis type.