Idiopathic Pulmonary Alveolar Proteinosis Research Articles

Experience with Treatment of Pulmonary Alveolar Proteinosis from a Tertiary Care Centre in North India

Pulmonary alveolar proteinosis (PAP) is a disorder characterised by accumulation of lipids and proteins in the alveoli, with the resultant symptoms ranging from indolent subclinical disease to progressive respiratory failure. We retrospectively studied five patients with PAP managed at our center between January 2007 and April 2010, with whole lung lavage (WLL) and/or subcutaneous granulocyte macrophage-colony stimulating factor (GM-CSF) therapy. Patients undergoing WLL under general anaesthesia were supplemented with three months of GM-CSF therapy. Pre- and post-lavage symptom assessment was performed with a 10-point, symptom-based visual analogue scale. Their mean age was 37.6-7.0 years; there were four males. Diagnosis of PAP [idiopathic (n=3); secondary to Nocardia (n=1)] was established by surgical lung biopsy in four patients who presented with respiratory failure. Three patients with idiopathic PAP (n=3) were treated with a combination of GM-CSF and WLL; one patient with secondary PAP was treated with antibiotics alone. In another patient transbronchial lung biopsy was used to diagnose PAP and GM-CSF alone was administered. All patients were followed up for a median period of two years (range 0.5-3 years). Significant improvement was achieved in all the patients with therapeutic WLL and/or GM-CSF. Whole lung lavage appeared to be an effective and safe therapy in patients with PAP. Efficacy of simultaneous administration of GM-CSF and WLL in the treatment of PAP merits further study.

High-affinity autoantibodies specifically eliminate granulocyte-macrophage colony-stimulating factor activity in the lungs of patients with idiopathic pulmonary alveolar proteinosis

AbstractDeficiency of granulocyte-macrophage colony-stimulating factor (GM-CSF) in mice results in pulmonary alveolar proteinosis (PAP) from impaired surfactant catabolism by alveolar macrophages (AMs). Recently, we have shown that neutralizing anti-GM-CSF autoantibodies develop specifically in patients with idiopathic pulmonary alveolar proteinosis (iPAP). Analogous to murine PAP models, it is plausible that the autoantibodies reduce GM-CSF activity, resulting in AM dysfunction and surfactant accumulation. To examine this hypothesis, we estimated the neutralizing activity of the autoantibodies in the lungs of patients and characterized their biologic properties. GM-CSF bioactivity was completely abrogated in the bronchoalveolar lavage fluid (BALF) of patients with iPAP but not in healthy subjects. Autoantibodies were present in the alveoli in high concentrations and colocalized with GM-CSF. They recognized human GM-CSF with high avidity (KAV = 20.0 ± 7.5 pM) and high specificity, reacting with its superstructure and neutralizing GM-CSF activity to a level 4000 to 58 000 times the levels of GM-CSF normally present in the lung. Although target epitopes varied among patients, GM-CSF amino acids 78 to 94 were consistently recognized. Thus, autoantibodies bind GM-CSF with high specificity and high affinity, exist abundantly in the lung, and effectively block GM-CSF binding to its receptor, inhibiting AM differentiation and function. Our data strengthen the evidence associating anti-GM-CSF autoantibodies with the pathogenesis of this disease. (Blood. 2004;103:1089-1098)

HIGH-RESOLUTION COMPUTED TOMOGRAPHY IN ALVEOLAR PROTEINOSIS

TYPE: Abstract Publication TOPIC: Imaging PURPOSE: Although the crazy-paving pattern on computed tomography is characteristic for pulmonary alveolar proteinosis (PAP), it is not specific and has not been compared between idiopathic and secondary PAPs in the large studies. The aim of this study was to determine the high resolution computed tomography (HRCT) features of idiopathic PAP. METHODS: HRCT images of 35 patients (mean age: 38±14years; 54.3% male) with idiopathic PAP (proved by bronchoalveolar lavage or biopsy) were reviewed by two experienced pulmonary radiologist and detailed findings were reported. RESULTS: The predominant HRCT presentation of PAP was interlobular septal thickening (ILST;100%) and ground glass opacities (GGOs; 91.7%), resulting in crazy-paving pattern (83%). All patients had diffuse bilateral lung involvement that was symmetric in 97%. ILST and GGO without crazy-paving were seen in 17% and 14.7%, respectively. The overall extent of parenchymal involvement was 50 to 75% in 80% of patients. Thirty three cases (94%) had areas of geographic sparing within the affected lung. Peripheral sparing was seen in 85.7% of patients, including three patterns with some overlap: costophrenic angle (80%), apices (60%), and subpleural (57%) sparing. Other HRCT findings were: consolidation (63%), pulmonary nodules (31.4%), mediastinal and/or hilar lymphadenopathy (23%), mass-like consolidation (17%), pleural effusion (8.6%), and honey combing (5.7%). All female patients (n=16) had crazy-paving, while 13 out of 19 (68%) male patients had crazy-paving on their lung HRCT (p=0.02). CONCLUSIONS: This study demonstrated that the predominant HRCT presentation of idiopathic PAP was interlobular septal thickening and ground glass opacities, resulting in crazy-paving pattern. CLINICAL IMPLICATIONS: resulting in crazy-paving pattern. DISCLOSURE: No significant relationships. KEYWORD: Pulmonary Alveolar Proteinosis (PAP), Idiopathic PAP, Crazy-Paving, Computed Tomography

Natural Autoantibodies in Chronic Pulmonary Diseases.

In autoantibody-mediated autoimmune diseases, pathogenic autoantibodies generated by a failure of central or peripheral tolerance, have different effects mediated by a variety of mechanisms. Interestingly, even non-autoimmune chronic diseases have a set of disease-specific natural autoantibodies that are maintained for a long time. Because most of these natural autoantibodies target intracellular proteins or long non-coding RNAs, they are speculated to be non-pathological and have some important as yet unrecognized physiological functions such as debris clearance. Recently, we revealed a set of disease-specific natural autoantibodies of chronic pulmonary diseases with unknown etiology by protein arrays that enable detection of specific autoantibodies against >8000 targets. Surprisingly, some of the targeted antigens of disease-specific autoantibodies were subsequently reported by other laboratories as strongly associated with the disease, suggesting that these antigens reflect the pathology of each disease. Furthermore, some of these autoantibodies that target extracellular antigens might modify the original course of each disease. Here, we review the disease-specific natural autoantibodies of chronic pulmonary diseases, including chronic fibrosing idiopathic interstitial pneumonias, sarcoidosis, and autoimmune pulmonary alveolar proteinosis, and discuss their utility and effects.

Therapeutic effect of subcutaneous injection of low dose recombinant human granulocyte-macrophage colony-stimulating factor on pulmonary alveolar proteinosis

ObjectiveTo observe the efficacy of recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) for pulmonary alveolar proteinosis (PAP).Materials and methodsA total of 55 patients with PAP were screened at Shanghai Pulmonary Hospital between May 2014 and May 2018. Among these, 42 were diagnosed with idiopathic PAP, 24 were included in this study, 20 were treated for 6 months, and 17 were followed up for additional 6 months. All patients received a subcutaneous injection of 75μg/d GM-CSF qd for 1 month. The therapeutic dose was adjusted according to the changes in the lesions of chest CT. If the lesions were absorbed, subcutaneous injections of 75μg/d GM- CSF qd and 75μg/d GM-CSF qod were given for 2 and 3 months, otherwise, the dose was increased to 150μg/d GM-CSF qd and 150μg/d qod for 2 and 3 months, respectively. All cases were treated once a day in the first 3 months and once every other day in the last 3 months. The total course of treatment was 6 months. After withdrawal, the patients were followed up for another 6 months. The deadline of follow up was September 30, 2019.ResultsTwenty patients completed the treatment and efficacy evaluation. One patient was completely cured, 16 cases improved, three cases were noneffective. After 1-month evaluation, 12 patients received an increased dose (150μg) from the second month of treatment. Seventeen patients completed the 12-month follow-up, among which fourteen improved. CT showed the lesions were slightly increased in three cases. Economic burden was the following: RMB 7324–15,190 Yuan were required for the 6-month treatment course, which is significantly lower compared to other treatment methods.ConclusionSubcutaneous injection of rhGM-CSF at low dose (75μg-150μg /d) is effective treatment for patients with idiopathic PAP.Trial registrationNCT01983657. Registered 16 April 2013.

Pulmonary Alveolar Proteinosis- A Case Report

ABSTRACT Background: Pulmonary Alveolar Proteinosis (PAP) is a rare pulmonary disorder caused by a congregation of excessive lipoproteinaceous material in the alveolar spaces due to impaired surfactant metabolism. The congregation of the protein in the alveolar space leads to difficulty in breathing, impaired pulmonary immunity, and susceptibility to both opportunistic and acquired pulmonary infections. Although Pulmonary Alveolar Proteinosis is rare, there are potential treatments. Whole-lung lavage is the most widely accepted therapy and course of treatment. An additional form of therapy, GM-CSF stimulating therapy, uses recombinant deoxyribonucleic acid (DNA) technology to increase white cell production. Purpose: The purpose of this case report is to follow a patient through whole-lung lavage therapy to determine outcome and clinical improvement. Case Description: This case report follows a 55-year-old female patient diagnosed with secondary, idiopathic Pulmonary Alveolar Proteinosis through eight normal saline whole-lung lavages. The patient’s treatment was led by a pulmonologist with previous Pulmonary Alveolar Proteinosis experience. Outcome: By the end of the first four lavages, the patient showed clinical improvement, but during a two-month break from therapy, symptoms returned. Following the break, the patient underwent four additional lung lavage sessions and experienced similar relief as in previous courses of treatment. Discussion: The patient did complete an additional eight lavages to wash out excess lipoproteinaceous material to provide a longer period of symptom relief. The additional form of therapy, GM-CSF, is not a therapy option for this patient as the disease was idiopathic in nature. The last available treatment option for this patient is a lung transplant.

ABCG1 regulates pulmonary surfactant metabolism in mice and men

Idiopathic pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of surfactant. Surfactant synthesis and secretion are restricted to epithelial type 2 (T2) pneumocytes (also called T2 cells). Clearance of surfactant is dependent upon T2 cells and macrophages. ABCG1 is highly expressed in both T2 cells and macrophages. ABCG1-deficient mice accumulate surfactant, lamellar body-loaded T2 cells, lipid-loaded macrophages, B-1 lymphocytes, and immunoglobulins, clearly demonstrating that ABCG1 has a critical role in pulmonary homeostasis. We identify a variant in the ABCG1 promoter in patients with PAP that results in impaired activation of ABCG1 by the liver X receptor α, suggesting that ABCG1 basal expression and/or induction in response to sterol/lipid loading is essential for normal lung function. We generated mice lacking ABCG1 specifically in either T2 cells or macrophages to determine the relative contribution of these cell types on surfactant lipid homeostasis. These results establish a critical role for T2 cell ABCG1 in controlling surfactant and overall lipid homeostasis in the lung and in the pathogenesis of human lung disease.

Advance in pathogenesis and treatment of idiopathic pulmonary alveolar proteinosis

Advance in pathogenesis and treatment of idiopathic pulmonary alveolar proteinosis

Effectiveness and safety of inhaled granulocyte-macrophage colony-stimulating factor therapy in idiopathic pulmonary alveolar proteinosis

To investigate the effectiveness and safety of inhaled granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy in idiopathic pulmonary alveolar proteinosis (PAP). Ten PAP patients were enrolled, who were hospitalized in the Affiliated Drum Tower Hospital of Nanjing University Medical School from January 2012 to January 2014.All the patients were treated with inhaled GM-CSF therapy, high-dose therapy for 12 weeks, GM-CSF 150 µg twice a day on days 1-7, none for days 8-14, 6 cycles, low-dose therapy for 12 weeks, GM-CSF 150 µg inhaled once a day on days 1-7, none for days 8-14, 6 cycles, and followed-up for one year. Physiologic, serologic and radiologic features of the patients were analyzed. After inhaled therapy, clinical symptoms, oxygenation indexes, pulmonary function of nine patients were improved, and high resolution CT (HRCT) showed ground-glass lesions reduced. After inhaled therapy for 6 months, the average level of arterial oxygen partial pressure (PaO₂) was significantly higher than that before therapy ((75.5 ± 7.0) vs (63.6 ± 8.9) mmHg (1 mmHg=0.133 kPa)), while alveolar-arterial oxygen pressure difference (P(A-a)O₂) was lower than before ((25.1 ± 7.1) vs (41.2 ± 13.5) mmHg) (both P<0.01). Similarly, vital capacity (VC)% predicted, forced vital capacity (FVC)% predicted and carbon monoxide diffusing capacity (DLCO)% predicted all improved after therapy ((77.3 ± 16.6)% vs (63.3 ± 16.6)%), (79.5 ± 17.6)% vs (64.9 ± 17.1)%), (69.4 ± 23.0)% vs (50.0 ± 19.9)%) (all P<0.01). The score of HRCT reduced after therapy for six months (P<0.05). While the levels of serum lactate dehydrogenase (LDH), carcinoembryonic antigen (CEA), CYFRA211 were unchanged. No serious adverse events occurred during observation. Inhaled GM-CSF therapy is safe and effective.

Features of idiopathic pulmonary alveolar proteinosis in high resolution computed tomography

SummaryBackgroundAlthough the crazy-paving pattern on computed tomography is characteristic for pulmonary alveolar proteinosis (PAP), it is not specific and has not been compared between idiopathic and secondary PAPs in the large studies. The aim of this study was to determine the high resolution computed tomography (HRCT) features of idiopathic PAP.Material/MethodsHRCT images of 35 patients (mean age: 38±14years; 54.3% male) with idiopathic PAP (proved by bronchoalveolar lavage or biopsy) were reviewed by two experienced pulmonary radiologist and detailed findings were reported.ResultsThe predominant HRCT presentation of PAP was interlobular septal thickening (ILST;100%) and ground glass opacities (GGOs; 91.7%), resulting in crazy-paving pattern (83%). All patients had diffuse bilateral lung involvement that was symmetric in 97%. ILST and GGO without crazy-paving were seen in 17% and 14.7%, respectively. The overall extent of parenchymal involvement was 50 to 75% in 80% of patients. Thirty three cases (94%) had areas of geographic sparing within the affected lung. Peripheral sparing was seen in 85.7% of patients, including three patterns with some overlap: costophrenic angle (80%), apices (60%), and subpleural (57%) sparing. Other HRCT findings were: consolidation (63%), pulmonary nodules (31.4%), mediastinal and/or hilar lymphadenopathy (23%), mass-like consolidation (17%), pleural effusion (8.6%), and honey combing (5.7%). All female patients (n=16) had crazy-paving, while 13 out of 19 (68%) male patients had crazy-paving on their lung HRCT (p=0.02).ConclusionsThis study demonstrated that the predominant HRCT presentation of idiopathic PAP was interlobular septal thickening and ground glass opacities, resulting in crazy-paving pattern.

FRI0313 Detection of anti-granulocyte macrophage colony-stimulating factor autoantibody in systemic lupus erythematodes

BackgroundSystemic lupus erythematodes(SLE) causes various symptoms in the respiratory system. Ground glass opacity (GGO) with SLE is conventionally considered interstitial pneumonia caused by the deposition of immune complexes. However, in...

Characterization of pathogenic human monoclonal autoantibodies against GM-CSF

The origin of pathogenic autoantibodies remains unknown. Idiopathic pulmonary alveolar proteinosis is caused by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). We generated 19 monoclonal autoantibodies against GM-CSF from six patients with idiopathic pulmonary alveolar proteinosis. The autoantibodies used multiple V genes, excluding preferred V-gene use as an etiology, and targeted at least four nonoverlapping epitopes on GM-CSF, suggesting that GM-CSF is driving the autoantibodies and not a B-cell epitope on a pathogen cross-reacting with GM-CSF. The number of somatic mutations in the autoantibodies suggests that the memory B cells have been helped by T cells and re-entered germinal centers. All autoantibodies neutralized GM-CSF bioactivity, with general correlations to affinity and off-rate. The binding of certain autoantibodies was changed by point mutations in GM-CSF that reduced binding to the GM-CSF receptor. Those monoclonal autoantibodies that potently neutralize GM-CSF may be useful in treating inflammatory disease, such as rheumatoid arthritis and multiple sclerosis, cancer, and pain.

Biochemical index and immunological function in the peripheral blood of patients with idiopathic pulmonary alveolar proteinosis

Idiopathic pulmonary alveolar proteinosis (PAP) has recently been recognized as a disease of impaired alveolar macrophage function caused by the neutralizing granulocyte-macrophage colony stimulating factor (GM-CSF) autoantibody. However, the change of immunological function and biochemical index in the peripheral blood of patients with idiopathic PAP remains unclear. The clinical data of 29 patients with idiopathic PAP and 30 normal subjects were retrospectively analyzed. Biochemical indices, immunoglobulin and complement of all participants, and immunocytes in 19 patients and 30 normal subjects were evaluated. The peripheral blood of the patients showed a decrease in CD4+/CD8+ (P<0.05) and the percentage of the CD4+ T lymphocyte and helper T lymphocyte (both P<0.01), whereas an increase was observed in the percentage of the suppressor T lymphocyte (P<0.01) compared to the normal subjects. No significant differences were found in the concentration of IgG, IgA, IgM, C3 and C4 between the two groups. An increase was observed in LDH (P<0.01) and cytokeratin fragment antigen (CYFR) 211 (P<0.01) in the peripheral blood of the patients compared to that of normal subjects. The serum level of LDH was negatively correlated with FVC% Pred (P<0.05), DLCO% Pred (P<0.05), PaO2 (P<0.05) and positively associated with the dyspnea score (P<0.05) of 29 patients, and positively correlated with the score of high-resolution computed tomography (HRCT) of the chest of 21 patients (P<0.05). The serum level of CYFR211 was negatively correlated with DLCO% Pred (P<0.05) and positively associated with the dyspnea score (P<0.05). Findings of the present study suggest that hypofunction of cellular immunity may exist in the patients with idiopathic PAP. LDH and CYFRA211 may be considered as important indices to monitor the severity of idiopathic PAP.

Clinical significance of serum lipids in idiopathic pulmonary alveolar proteinosis

BackgroundIt is well known that pulmonary alveolar proteinosis(PAP) is characterised by accumulation of surfactant lipids and proteins within airspaces. However, few previous data describe the serum lipid levels associated with PAP.Materials and methodsWe retrospectively reviewed 25 patients with idiopathic PAP(iPAP). The serum lipid levels of patients with idiopathic PAP were compared with those of the healthy volunteers. In patients and healthy subjects, the LDL-C/HDL-C ratios were 2.94 ± 1.21 and 1.60 ± 0.70, respectively (p < 0.001), HDL-C were 1.11 ± 0.27 and 1.71 ± 0.71 respectively (p < 0.001). The values of LDL-C correlated significantly with those of PaO2 and PA-aO2 (r = -0.685, p = 0.003, and r = 0.688, p = 0.003, respectively). The values of LDL-C/HDL-C ratios also correlated with PaO2 levels and PA-aO2 levels (r = -0.698, p = 0.003, and r = 0.653, p = 0.006, respectively). 11 and 13 patients experienced respectively a decline in TC and LDL-C levels following whole lung lavage(WLL), the median decline was 0.71 mmol/L(p < 0.009) and 0.47 mmol/L(p < 0.003), respectively.Conclusionsthe serum lipid levels, especially the levels of LDL-C and LDL-C/HDL-C, may reflect the severity of the disease in PAP patients, and predict the therapeutic effect of WLL.

A Case of Pulmonary Alveolar Proteinosis Improved with Inhaled Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF)

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of lipoproteinaceous material within the alveoli. Several studies have recently found that autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) play a major role in the pathogenesis of idiopathic PAP. Consequently, inhaled or systemic injection of GM-CSF has been suggested as a promising treatment for PAP. A 54-year-old male visited our hospital for progressive dyspnea. Four years earlier, he was diagnosed with PAP based on a surgical lung biopsy in another institution. Whole-lung lavage was performed four times before he visited our hospital. We administered high-dose inhaled GM-CSF therapy for 12 weeks followed by 12 weeks of low-dose therapy. After the GM-CSF treatment, the patient’s symptoms, lung function, and radiological findings were improved significantly. (Korean J Med 2012;82:357-361)

Clinical Importance of Bronchoalveolar Lavage Fluid and Blood Cytokines, Surfactant Protein D, and Kerbs von Lungren 6 Antigen in Idiopathic Pulmonary Alveolar Proteinosis

To investigate the clinical importance of cytokines, surfactant protein D (SFTPD, formerly SP-D), and Kerbs von Lungren 6 antigen (KL-6) in bronchoalveolar lavage fluid (BALF) and blood in patients with idiopathic pulmonary alveolar proteinosis (iPAP). Patients with iPAP diagnosed by characteristic cytopathologic examination of BALF and pathologic examination of transbronchial lung biopsy specimens or open lung biopsy specimens were enrolled in the study from January 1, 1995, through June 30, 2005. To investigate the clinical importance of cytokines, SFTPD, and KL-6 in iPAP, we measured tumor necrosis factor superfamily, member 2 (TNF, formerly TNF-alpha) and interleukin (IL) 1beta in BALF and IL-6, IL-10, IL-8, chemokine (C-C motif) ligand 4 (CCL4, formerly MIP-1beta), chemokine (C-C motif) ligand 2 (CCL2, formerly MCP-1), SFTPD, and KL-6 in both BALF and blood in 15 patients with iPAP and 48 patients with interstitial lung diseases (diseased controls) (including 20 with interstitial pneumonitis associated with collagen vascular diseases, 13 with idiopathic pulmonary fibrosis, and 15 with sarcoidosis) and 20 individuals without pulmonary diseases (lung controls). Patients with iPAP had significantly higher levels of TNF, IL-6, IL-8, CCL4, CCL2, SFTPD, and KL-6 in BALF than did lung controls and had significantly higher levels of CCL4, CCL2, SFTPD, and KL-6 levels in BALF than did diseased controls. Patients with iPAP had significantly higher levels of IL-10, IL-8, CCL2, SFTPD, and KL-6 in blood than did lung controls and significantly higher levels of KL-6 in blood than did diseased controls. Levels of KL-6 both in BALF and blood were significantly correlated with serum lactate dehydrogenase level, arterial oxygen tension, and alveolar-arterial gradient in partial pressure of oxygen, the severity markers for iPAP, and were significantly higher in patients with iPAP who required subsequent therapeutic lung lavage. Patients with iPAP had elevated levels of SFTPD, KL-6, and cytokines in both BALF and blood. Elevated levels of KL-6 in both BALF and blood may be valuable in reflecting disease severity of patients with iPAP and in determining the need for therapeutic lung lavage.

CS05-2. Genetic and functional analysis of a panel of truly human monoclonal antibodies against GM-CSF generated from idiopathic pulmonary alveolar proteinosis (IPAP) patients and potential therapeutic uses as an anti-inflammatory agent

CS05-2. Genetic and functional analysis of a panel of truly human monoclonal antibodies against GM-CSF generated from idiopathic pulmonary alveolar proteinosis (IPAP) patients and potential therapeutic uses as an anti-inflammatory agent

Impaired lipid metabolism in idiopathic pulmonary alveolar proteinosis.

BackgroundIt is well known that lipids abnormally accumulate in the alveoli during idiopathic pulmonary alveolar proteinosis (PAP). It is unclear, however, whether lipids also abnormally accumulate in serum. This study investigated the serum lipid panels in idiopathic PAP patients and explored the relationships between serum levels and the severity of idiopathic PAP.Methods and ResultsClinical data including the level of serum lipids were evaluated in 33 non-diabetic idiopathic PAP patients and 157 healthy volunteers. Serum levels of triglyceride were higher in PAP patients than in healthy subjects (median: 192.00 mg/dl (P25: 104.36, P75: 219.00) vs 119.56 mg/dl (P25: 78.81, P75: 193.03), P < 0.05), while high-density lipoprotein cholesterol (HDL-C) levels were lower in patients than in the control group (42.50 ± 10.30 vs 51.34 ± 12.06 mg/dl, P < 0.01). Forced expiratory volume in one second and forced vital capacity in hypertriglyceridemia patients were lower than those in patients with normal triglyceride. Serum LDL-C and HDL-C ratio correlated negatively with PaO2 (r = -0.403, P < 0.05) and positively with lactate dehydrogenase (r = 0.381, P < 0.05).ConclusionsPAP associates with high triglyceride and low HDL levels in the serum, and these lipids provide potential intervention strategy for treatment.

A Case of Idiopathic Pulmonary Alveolar Proteinosis in a Motorcycle Mechanic

A Case of Idiopathic Pulmonary Alveolar Proteinosis in a Motorcycle Mechanic

Pulmonary alveolar proteinosis and successful therapy with combined lavage procedures: Case reports

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of lipoproteinaceous material within alveolar spaces. Whole-lung lavage (WLL) has been the most common therapeutic intervention for this disorder. However, patients presenting with PAP are usually hypoxemic or in poor clinical condition, and WLL may be impossible to perform. In such cases, multiple segmental lavage (MSL) may be advocated as a first-choice therapy prior to WLL. Herein, we present two cases with idiopathic PAP treated successfully with both lavage techniques consecutively. After the MSL procedure, WLL was performed, and both patients showed a marked clinical and physiologic improvement. Therefore, for patients who are not good candidates for general anesthesia, we recommend MSL (or 'prewash') before WLL to produce an increase in the blood oxygen level for long-duration general anesthesia. In the surgical room, close monitoring and repositioning of the patient as well as maintenance and inspection of the correct tube position, and manual chest wall percussion are extremely important for the safety and success of the procedure.