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

Pulmonary alveolar proteinosis is a rare disorder that is characterized by the abundant accumulation of surfactant-derived material within the alveolar spaces and distal bronchioles. Although the findings of homogenous ground-glass opacities overlaid by thickened septa, which form a "crazy paving" pattern on high-resolution computed tomography, can assist in making a diagnosis, a lung biopsy is usually required. This disorder has a variable clinical course, from spontaneous resolution to respiratory failure and death. While a whole lung lavage has been the standard treatment since the early 1960s, GM-CSF therapy has been attempted based on the recently suggested pathogenetic mechanism. We report a case of pulmonary alveolar proteinosis that resolved spontaneously after an open lung biopsy.
Biopsy ; Bronchioles ; Bronchoalveolar Lavage ; Diagnosis ; Granulocyte-Macrophage Colony-Stimulating Factor ; Lung ; Pulmonary Alveolar Proteinosis* ; Respiratory Insufficiency

Pulmonary alveolar proteinosis(PAP) is a rare disease in which the alveolar spaces are filled with an eosinophilic, PAS-positive material, whereas the interstitial architecture of the lung usually remains unaffected. Although a definitive diagnosis is usually made by an open lung biopsy, bronchoalveolar lavage(BAL) cytology may play a decisive role in the diagnosis and therapy of these patients and may spare a patient a more invasive diagnostic procedure. The author presents a patient in whom BAL cytology specimen contained the characteristic globules of amorphous proteinaceous PAS-positive material accompanied by background of rare macrophages and inflammatory cells. Ultrastructural study using BAL specimen can confirm the diagnosis of PAP.
Biopsy ; Bronchoalveolar Lavage* ; Diagnosis ; Eosinophils ; Humans ; Lung ; Macrophages ; Pulmonary Alveolar Proteinosis* ; Rare Diseases

OBJECTIVE: To evaluate the diagnostic value and limits of transbronchial lung biopsy (TBLB) in pulmonary alveolar proteinosis (PAP). METHODS: The complete hospital data from Second Xiangya Hospital, Central South University, between June, 2006 and December, 2012, were analyzed retrospectively in 25 patients with PAP (who were diagnosed pathologically by TBLB or not by TBLB) and in 4 patients with other disease (who were misdiagnosed by TBLB). RESULTS: Among the 25 patients with PAP, 14 patients were confirmed by TBLB in the fi rst time [TBLB positive rate in the first time was 56% (14/25)]; 6 patients who were misdiagnosed by TBLB in the fi rst time were confirmed by TBLB in the second time [the positive rate in the second time was 24% (6/25)]. Th e total positive rate was 80% (20/25). Th e total negative rate was 20% (5/25). Five patients with PAP, who showed negative results in TBLB analysis, were confirmed by the typical CT as well as the whole lung lavage. In addition, 4 patients with other diseases were misdiagnosed as PAP by TBLB. CONCLUSION TBLB is a very good diagnosis method. But the negative results in the first time cannot exclude PAP. TBLB should be repeated if conditions allow. If clinical manifestation and CT results are typical, it can be used for diagnosis of PAP combined with the positive results from bronchoalveolar lavage fluid check. However, for non-typical cases, it needs TBLB pathological diagnosis.
Biopsy ; Diagnostic Errors ; Humans ; Lung ; pathology ; Pulmonary Alveolar Proteinosis ; diagnosis ; pathology ; Retrospective Studies

We had performed six times (4 in right lung, 2 in left lung) bronchoalveolar lavages in a patient who has hypertension and suffered cerebral vascular accident previously. The diagnosis of PAP was confirmed by an open lung biopsy and multiple bronchoalveolar lavages were done for two and a half months. Even though she showed slight improvement in chest radiographs, she showed minimal improvement in her clinical course until the third lavage was done. She had to receive tracheostomy and prolonged ventilatory care because her PAP was progressed rapidly and complicated with superinfection. In addition, she sustained pneumothorax as a complication of the prolonged ventilatory care and the lavages. She was discharged one month after the last lavage and her clinical course has been uneventful 10 months thereafter.
Biopsy ; Bronchoalveolar Lavage* ; Diagnosis ; Humans ; Hypertension ; Lung ; Pneumothorax ; Pulmonary Alveolar Proteinosis* ; Radiography, Thoracic ; Superinfection ; Therapeutic Irrigation ; Tracheostomy

Adult ; Humans ; Indium ; adverse effects ; Male ; Occupational Exposure ; adverse effects ; Pulmonary Alveolar Proteinosis ; chemically induced ; diagnosis ; surgery

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

Membranous glomerulonephritis is one of the most common causes of nephrotic syndrome in adults. Pulmonary alveolar proteinosis (PAP) is a rare lung disease in which abnormal accumulation of surfactant occurs within the alveoli. We describe a 61-year-old man with concurrent membranous glomerulonephritis and PAP, which is very rare; both are pathophysiologically related to an abnormal immune response. A patient came to hospital with leg edema but no respiratory symptoms. Chest X-ray and CT showed classical PAP findings, which are ground-glass opacities with interlobular septal thickening, in both lung fields. A bubbly whitish secretion retrieved via broncho-alveolar lavage showed neutrophils and lymphocytes as well as Periodic acid-Schiff-positive proteinaceous materials. A kidney biopsy revealed findings of membranous glomerulonephritis with irregular subepithelial deposits by electron microscopy. At 1 year after diagnosis, the membranous glomerulonephritis was well under control with steroids and mycophenolate mofetil but PAP became aggravated gradually and whole-lung lavage was needed.
Adult ; Biopsy ; Diagnosis ; Edema ; Glomerulonephritis, Membranous* ; Humans ; Kidney ; Leg ; Lung ; Lung Diseases ; Lymphocytes ; Microscopy, Electron ; Middle Aged ; Nephrotic Syndrome ; Neutrophils ; Pulmonary Alveolar Proteinosis* ; Steroids ; Therapeutic Irrigation ; Thorax

BACKGROUND: A CT scan is a useful modality for the diagnosis and evaluation of disease activity in patients with pulmonary tuberculosis. However, the CT diagnosis of pulmonary tuberculosis is sometimes difficult in patients with an atypical CT pattern, especially with lobar consolidation mimicking pneumonia. The aim of this study was to evaluate the clinical and CT features of pulmonary tuberculosis, simulating pneumonia, from a CT scan. MATERIALS AND METHODS: The clinical and CT features in 21 patients, where the CT diagnosis was pneumonia, or the CT differential diagnosis included pneumonia, were retrospectively analyzed. RESULTS: Of the 21 patients, 6 were immunocompromised, 15 presented with fever or leukocytosis and 15 showed positive sputum smear test for acid fast bacilli. Also, 17 of the 21 patients showed a positive sputum culture test. On the CT scan, consolidation was noted in all patients (100%), volume loss of the involved lobe or segment in 12 (57%), bronchogenic spread in 15 (71%), a cavity in 7 (33%) and bronchial wall thickening also in 7 (33%). The location of the consolidation revealed a relatively even distribution, with no specific predilection site. The other associated pulmonary diseases included ARDS, bronchiectasis, severe pulmonary emphysema, idiopathic pulmonary fibrosis and pulmonary alveolar proteinosis. CONCLUSION: In the immunocompromised patients, or patients with an underlying pulmonary disease, whose CT scans showed pulmonary consolidation, especially in association with findings of bronchogenic spread, a cavity or bronchial wall thickening, meticulous examination for pulmonary tuberculosis is recommended.
Bronchiectasis ; Diagnosis ; Diagnosis, Differential ; Fever ; Humans ; Idiopathic Pulmonary Fibrosis ; Immunocompromised Host ; Leukocytosis ; Lung ; Lung Diseases ; Pneumonia* ; Pulmonary Alveolar Proteinosis ; Pulmonary Emphysema ; Retrospective Studies* ; Sputum ; Tomography, X-Ray Computed ; Tuberculosis ; Tuberculosis, Pulmonary*