Pulmonary alveolar proteinosis in a patient with Behcet's disease

Secondary pulmonary alveolar proteinosis (PAP) has been described in several clinical settings that can be grouped into three main categories: infections of the lung; haematological malignancies and other conditions that alter the patient's immune status; and exposure to inhaled chemicals and minerals. Recent studies reported that anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibody was present in the serum of patients with idiopathic PAP but not in patients with secondary PAP or in normal subjects. The present report describes the interesting case of a patient with Behcet's disease and PAP. The absence of anti-GM-CSF antibodies in this patient suggested a diagnosis of secondary PAP.     

肺泡蛋白沉积症研究进展

肺泡蛋白沉积症(PAP)是一种肺部少见病,以肺泡腔和细支气管腔沉积PAS染色阳性的不可溶性富磷脂蛋白质物质为特征.可分为因表面活性蛋白或GM-CSF受体基因突变导致的先天性PAP、因GM-CSF抗体阻断了肺泡巨嗟细胞的激活导致的自身免疫性PAP和继发于血液系统疾病或吸入有毒气体导致的继发性PAP.临床表现主要为进行性气促、低氧血症,胸部高分辨率CT主要表现为毛玻璃样高密度影和“疯狂堆砌”改变,支气管肺泡灌洗液PAS染色阳性可确诊.肺泡灌洗治疗有效,GM-CSF替代疗法和针对GM-CSF抗体的治疗是正在研究的治疗方法.     

胸部高分辨CT在肺泡蛋白沉积症分级诊断中的应用价值

目的从胸部影像学角度探讨肺泡蛋白沉积症(PAP)的诊断分级,以指导治疗。方法对上海市肺科医院2000年1月至2010年12月收治的31例确诊PAP患者胸部高分辨CT(HRCT)进行分级,选择4个代表层面(主动脉弓、隆突、左或右下肺静脉汇合层面和膈上层面),病灶在这些层面的所占范围进行5级评分,结合临床症状、肺功能指标,建立一套PAP胸部HRCT诊断分级标准,根据诊断分级提出相应的治疗决策。结果 (1)按胸部HRCT病灶范围将PAP患者分为4级:1级(≤8分)3例;2级(>8～16分)12例;3级(>16～24分)6例;4级(>24分)10例。(2)PAP患者胸部HRCT评分与呼吸困难评分、症状总评分呈正相关(r=0.748、0.578,P均<0.01)。(3)PAP患者胸部HRCT评分与用力肺活量(FVC)占预计值%、第一秒用力呼气容积(FEV1)占预计值%、一氧化碳弥散量占预计值%及动脉血氧分压(PaO2)均呈负相关(r=-0.486、-0.376、-0.596、-0.444,P<0.01或0.05)。(4)结合胸部HRCT分级和PaO2将PAP患者分为4期:1期:HRCT分级1级伴PaO2≥8.0 kPa;2期:HRCT分级2级伴PaO2≥8.0 kPa;3期:HRCT分级3级伴PaO2≥8.0 kPa;4期:HRCT分级4级,或HRCT分级2～3级伴PaO2<8.0 kPa。(5)不同PAP患者胸部HRCT诊断分级建议:1、2期建议对症治疗及长期随访胸部HRCT;3期患者建议序贯肺泡灌洗或GM-CSF治疗;4期患者建议全肺灌洗。结论胸部HRCT诊断分级可以作为评估PAP患者病情严重程度的基础,并在此基础上选择合适的治疗方法,对临床上诊断和治疗PAP具有一定指导意义。     

Hemodynamic changes during whole bronchoalveolar lavage in two cases of pulmonary alveolar proteinosis

Fove whole bronchoalveolar lavages were performed in 2 patients with pulmonary alveolar proteinosis with continuous monitoring of mixed venous and arterial oxygen saturation. Hemodynamic parameters and gas-exchange status were measured during the different phases of the lavage. In the phase of filled lung, a significant increase of arterial partial pressure (PaO₂) and arterial saturation of oxygen were observed, secondary to a decrease in the intrapulmonary shunt. The mean pulmonary arterial pressure, pulmonary vascular resistances and cardiac index were higher during the filling of the lung as compared to the controls. During the empty lung phase, although PaO₂ decreased (without reaching statistical significance), due to an increase in the intrapulmonary shunt, the increase in cardiac output during this phase left the oxygen delivery (DO₂) unchanged.To be presented in part at the 1991 American Thoracic Society Congress     

Hyalinosis cutis et mucosae: gingival involvement

Hyalinosis cutis et mucosae (lipoid proteinosis, Urbach-Wiethe disease) is a rare syndrome with autosomal recessive inheritance. The disease is characterized by diffuse deposition of a hyaline-like substance in the dermis, the submucosal connective tissue and various internal organs. The oral mucosa of affected people becomes nodular and thickened, with primary involvement of the labial, buccal and palatal mucosa, posterior tongue, and lingual frenulum. We report the case of a 66-year-old man with hyalinosis cutis et mucosae who presented with gingival hyperplasia due to diffuse deposition of hyaline-like material, and discuss the diagnostic significance of the microscopic findings of the gingival tissues. The present clinical features are also compared to those of the same patient at the age of 38 years.     

Secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome

Pulmonary alveolar proteinosis （PAP） IS an uncommon disease first reported by Rosen et al^1 in 1958, and characterized by the accumulation of surtactant proteins and phospholipids within the alveolar spaces. Acquired PAP is divided into two forms based on clinical features： idiopathic PAP and secondary PAE Secondary PAP is reported to be associated with haematological malignancies, Pneumocystis carinii pneumonia and inhalation of silica or titanium, and the most frequent underlying disease of secondary PAP is haematological malignancy. The exact incidence of PAP in haematological malignancies is still obscure, since there have been only sporadic reports of secondary PAP.^ 2, 3     

Unilateral Autoimmune Pulmonary Alveolar Proteinosis with Polymyositis-related Interstitial Lung Disease

A 61-year-old patient with cystic bronchiectasis and bronchial artery hyperplasia in the left lung was diagnosed with polymyositis-related interstitial lung disease. After nine months of immunosuppressive therapy, he developed unilateral autoimmune pulmonary alveolar proteinosis (APAP) in the right lung with respiratory failure. After bronchial artery embolization to prevent massive hemoptysis, whole-lung lavage was performed using veno-venous extracorporeal membrane oxygenation. His respiratory condition improved, and he was discharged from the hospital with supplemental oxygen. Three reported cases of APAP with polymyositis-related interstitial lung disease, including the present case, were all positive for anti-glycyl tRNA synthetase antibody and were under immunosuppressive treatment.     

Ocular manifestations in lipoid proteinosis: A rare clinical entity

Lipoid proteinosis is a rare autosomal recessive genodermatosis with abnormal lipid protein complexes deposition in different parts of the body, especially in the skin and mucus membranes of the upper aerodigestive tract. Though ocular involvement in lipoid proteinosis is rare, ophthalmologists may encounter diverse ocular complications accompanying this syndrome in clinical practice. We describe a case of lipoid proteinosis involving bilateral eyelids with pathognomonic moniliform blepharosis in a 33-year-old gentleman who presented with the complaints of itching of eye lids on and off since 10 years.