Bronchoalveolar Lavage ; Humans ; Lung ; Pulmonary Alveolar Proteinosis/therapy*

Pulmonary alveolar proteinosis is such an extremely rare disease in Korea, that only a few cases have been reported. Meanwhile five cases were experienced at Seoul National University Hospital over ten years since 1987. We summarized the clinical characteristics and courses of them. Seven cases reported in the literature were included to add data about clinical characteristics and courses although only a few case reports mentioned patient's course. Middle aged male patients were mainly affected. No association with particular environmental or occupational exposure was identified. Dyspnea on exertion was the main symptom. Bilateral crackles were consistent, and bilateral parahilar hazy infiltrations on plain chest radiograph and ground glass opacity on high-resolution CT were characteristic. Superimposed infection was not identified in any patient at the time of diagnosis. Decreased diffusing capacity and hypoxia were present in almost every case. Whole lung lavage proved to be an effective therapeutic measure. The response to treatment was good. Long-term course of the disease, e.g. recurrence rate, is not yet known.
Adult ; Age Distribution ; Aged ; Female ; Hospitals, Public* ; Hospitals, University* ; Human ; Korea ; Male ; Middle Age ; Pulmonary Alveolar Proteinosis/therapy ; Pulmonary Alveolar Proteinosis/radiography ; Pulmonary Alveolar Proteinosis/physiopathology* ; Pulmonary Alveolar Proteinosis/diagnosis ; Sex Distribution ; Smoking/adverse effects

Adult ; Bronchoalveolar Lavage ; methods ; Humans ; Hypoxia ; etiology ; Male ; Pulmonary Alveolar Proteinosis ; complications ; therapy

Adult ; Bronchoalveolar Lavage ; methods ; Female ; Humans ; Male ; Middle Aged ; Pulmonary Alveolar Proteinosis ; therapy ; Treatment Outcome

Bronchoalveolar Lavage ; Extracorporeal Membrane Oxygenation ; Humans ; Male ; Middle Aged ; Pulmonary Alveolar Proteinosis ; therapy

OBJECTIVES: Pulmonary alveolar proteinosis (PAP) is a rare disease with non-specific and various clinical manifestations, often leading to misdiagnosis. This study aims to raise the awareness of this disease via summarizing the clinical characteristics, diagnosis, and therapy of PAP. METHODS: We retrospectively analyzed clinical data of 25 hospitalized cases of PAP during 2008 and 2019 in the Department of Respiratory and Critical Care Medicine of the Second Xiangya Hospital of Central South University. RESULTS: Cough with unkown reason and dyspnea were common clinical manifastations of PAP. Five patients had a history of occupational inhalational exposure. Sixteen patients had typical image features including ground-glass opacification of alveolar spaces and thickening of the interlobular and intralobular septa, in typical shapes called crazy-paving and geographic pattern. Fourteen patients underwent pulmonary function tests, revealing a reduction in the diffusing capacity for carbon monoxide. The positive rate of transbronchial biopsy was 95%. Five patients received the whole lung lavage and the symptoms and imaging fcauters significantly relieved after five-years follow-up. CONCLUSIONS PAP is characterized by radiographic pattern and pathology. Transbronchial lung biopsy is effective to make diagnosis of PAP. The whole lung lavage remains a efficient therapy.
Biopsy ; Bronchoalveolar Lavage ; Cough ; Dyspnea ; Humans ; Pulmonary Alveolar Proteinosis/therapy* ; Retrospective Studies

OBJECTIVETo study high resolution computed tomograghy (HRCT) features and changes of HRCT after whole lung lavage therapy in patients with pulmonary alveolar proteinosis (PAP).

METHODWe retrospectively reviewed the HRCT data of 23 times of lung lavage therapy in 16 PAP patients.

RESULTSHRCT scan showed bilateral clearly-defined patchy areas of ground-glass opacity and interlobular septal thickening, a pattern commonly characterized as "crazy paving". After whole lung lavage therapy, HRCT showed that the diffused ground-glass opacity palliated and the extent of lung opacity shrinked, while the reticular opacity and area of confluence persisted.

CONCLUSIONThe HRCT feature of PAP is relatively characteristic, and may be improved after whole lung lavage therapy.

Bronchoalveolar Lavage ; methods ; Female ; Humans ; Male ; Middle Aged ; Pulmonary Alveolar Proteinosis ; pathology ; therapy ; Retrospective Studies ; Tomography, X-Ray Computed ; methods

OBJECTIVETo describe the pathologic features and diagnostic algorithm of pulmonary alveolar proteinosis (PAP).

METHODSThirty-nine biopsy and postmortem cases of PAP were studied by light microscopy and histochemical staining using periodic acid-Schiff (with digestion) (PAS-D), mucicarmine (with digestion) (mucicarmine-D) and alcian blue.

RESULTSHistologically, the affected lung tissue displayed the following characteristic features: (1) alveoli and some of the small bronchioles were filled with eosinophilic and fine granular proteinaceous material with needle-like clefts; (2) proteinaceous material was seen admixed with various numbers of degenerated and sometimes exfoliated pneumocytes; (3) pneumocytes were hyperplastic; (4) alveolar capillaries and alveolar septa had become hyperemic, but pulmonary interstitial inflammation was not obvious; (5) no significant inflammation was identified in the bronchial wall; (6) compensating emphysema was noted in the surrounding lung parenchyma. Fragments of eosinophilic, finely granular proteinaceous material with needle-like clefts were also found in the bronchoalveolar lavage fluid under light microscopy. The proteinaceous material was stained red by PAS-D. The staining for mucicarmine-D was negative, while alcian blue staining was either weakly positive (faint blue staining) or negative. Pathologic examination of lung biopsies and bronchoalveolar lavage fluid thus remaines the gold standard for diagnosis of PAP.

CONCLUSIONSIdentification of homogeneous, eosinophilic, finely granular and PAS-D-positive proteinaceous material with needle-like clefts in alveolar spaces or bronchoalveolar lavage fluid is of diagnostic importance in PAP. Bronchoalveolar lavage, being a relatively safe and non-invasive procedure, can be a useful adjunct in arriving at the final conclusion.

Adult ; Bronchoalveolar Lavage ; Bronchoalveolar Lavage Fluid ; cytology ; Female ; Humans ; Lung ; pathology ; Male ; Middle Aged ; Periodic Acid-Schiff Reaction ; Pulmonary Alveolar Proteinosis ; pathology ; therapy ; Pulmonary Alveoli ; pathology

BACKGROUNDPulmonary alveolar proteinosis (PAP) is a rare lung disease, the most common type of which is autoimmune PAP. The gold standard therapy for PAP is whole lung lavage (WLL). Few studies have reported the optimal technique with which to evaluate the response to WLL. In this study, we aimed to identify parameters with which to assess the need for repeat WLL during a long-term 8-year follow-up.

METHODSWe conducted a retrospective analysis of 120 patients with autoimmune PAP with 80 of whom underwent WLL. Physiologic, serologic, and radiologic features of the patients were analyzed during an 8-year follow-up after the first WLL treatment.

RESULTSOf the 40 patients without any intervention, 39 patients either achieved remission or remained stable and only one died of pulmonary infection. Of the 56 patients who underwent WLL for 1 time, 55 remained free from a second WLL and 1 patient died of cancer. Twenty-four required additional treatments after their first WLL. The baseline PaO 2 (P = 0.000), PA-aO 2 (P = 0.000), shunt fraction rate (P = 0.001), percent of predicted normal diffusing capacity of the lung for carbon monoxide (DLCO%Pred) (P = 0.016), 6-min walk test (P = 0.013), carcinoembryonic antigen (CEA) (P = 0.007), and neuron-specific enolase (NSE) (P = 0.003) showed significant differences among the three groups. The need for a second WLL was significantly associated with PaO 2 (P = 0.000), CEA (P = 0.050) , the 6-minute walk test (P = 0.026), and DLCO%Pred (P = 0.041). The DLCO%Pred on admission with a cut-off value of 42.1% (P = 0.001) may help to distinguish whether patients with PAP require a second WLL.

CONCLUSIONSWLL is the optimal treatment method for PAP and provides remarkable improvements for affected patients. The DLCO%Pred on admission with a cut-off value of 42.1% may distinguish whether patients with PAP require a second WLL.

Adult ; Autoimmune Diseases ; diagnosis ; therapy ; Bronchoalveolar Lavage ; methods ; Female ; Follow-Up Studies ; Humans ; Lung ; pathology ; Male ; Middle Aged ; Pulmonary Alveolar Proteinosis ; diagnosis ; therapy ; Retrospective Studies ; Treatment Outcome

Idiopathic pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within alveoli. There were few reports on Asian populations with idiopathic PAP. We retrospectively reviewed 38 patients with idiopathic PAP in Korea. We assessed clinical features, therapeutic efficacy and outcomes of whole lung lavage in patients with idiopathic PAP. The mean age at diagnosis was 52 yr. Eighty six percent of patients were symptomatic at diagnosis. Dyspnea and cough were the most common symptoms. Crackles were the most common physical examination finding. On pulmonary function test, a mild restrictive ventilatory defect was common, with a predicted mean forced vital capacity (FVC) of 77% and forced expiratory volume in one second (FEV1) of 84.6%. Diffusing capacity was disproportionately reduced at 67.7%. Arterial blood gas analysis revealed hypoxemia with a decreased PaO2 of 69.0 mmHg and an increased D(A-a)O2 of 34.2 mmHg. After whole lung lavage, PaO2, D(A-a)O2 and DLCO were significantly improved, but FVC and total lung capacity (TLC) were not different. This is the first multicenter study to analyze 38 Korean patients with idiopathic PAP. The clinical features and pulmonary parameters of Korean patients with idiopathic PAP are consistent with reports in other published studies. Whole lung lavage appears to be the most effective form of treatment.
Adult ; Biopsy ; Bronchoalveolar Lavage ; Female ; Humans ; Korea ; Male ; Middle Aged ; Pulmonary Alveolar Proteinosis/diagnosis/pathology/*physiopathology/*therapy ; Respiratory Function Tests ; Retrospective Studies ; Survival Rate ; Treatment Outcome