Characterization and outcomes of pulmonary alveolar proteinosis in Brazil: a case series

Abstract

Objective:Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by the alveolar accumulation of surfactant, which is composed of proteins and lipids. PAP is caused by a deficit of macrophage activity, for which the main treatment is whole-lung lavage (WLL). We report the experience at a referral center for PAP in Brazil. Methods:This was a retrospective study involving patients with PAP followed between 2002 and 2016. We analyzed information regarding clinical history, diagnostic methods, treatments, and outcomes, as well as data on lung function, survival, and complications. Results:We evaluated 12 patients (8 of whom were women). The mean age was 41 ± 15 years. Most of the patients were diagnosed by means of BAL and transbronchial biopsy. The mean number of WLLs performed per patient was 2.8 ± 2.5. One third of the patients never underwent WLL. Four patients (33.3%) had associated infections (cryptococcosis, in 2; nocardiosis, in 1; and tuberculosis, in 1), and 2 (16.6%) died: 1 due to lepidic adenocarcinoma and 1 due to complications during anesthesia prior to WLL. When we compared baseline data with those obtained at the end of the follow-up period, there were no significant differences in the functional data, although there was a trend toward an increase in SpO2. The median follow-up period was 45 months (range, 1-184 months). The 5-year survival rate was 82%. Conclusions:To our knowledge, this is the largest case series of patients with PAP ever conducted in Brazil. The survival rate was similar to that found at other centers. For symptomatic, hypoxemic patients, the treatment of choice is still WLL. Precautions should be taken in order to avoid complications, especially opportunistic infections.

http://www.rarediseasesjournal.com/old-issues.php?journal=jrdrt&&v=4&&i=2&&y=2019&&m=April

“…The death causes were secondary infections such as tuberculosis or aspergillosis, as well as the progression of respiratory failure. The prognosis was worsened in case of late diagnosis with long-term unsuccessful antibacterial treatment in particular by tuberculostatics, as well as corticosteroids and physiotherapy, as it has been noted by other authors as well 13,14,33 .…”

Alveolar Proteinosis Secondary to M. tuberculosis, in a Patient with Transient CD4 Lymphocytopenia Due to Cryptococcus neoformans Infection: First Case in the Literature

“…The present case would be among the few cases reported since 1979 to date [13], in which alveolar proteinosis is described because of MBT infection. Literature describes two patterns: The first in which MBT infection is documented before the diagnosis of alveolar proteinosis with a negative report of anti-GM-CSF; and the second in which the measurement of anti-GM-CSF is not available, but antituberculous treatment leads to a decrease in proteinosis and favorable clinical outcomes [3][4][5][6][7][9][10][11]13,14]. Our case belongs to the second pattern of presentation; however, to our knowledge it would be the first case reporting alveolar proteinosis secondary to M. tuberculosis in a patient with transient CD4 lymphocytopenia and cryptococcosis.…”

“…trigger. The present would be among the first 15 cases to our knowledge describin association between M. tuberculosis and alveolar proteinosis [4][5][6][7][8][9][10][11][12][13][14][15].…”

“…Differentiating between the cause and the consequences of alveolar proteinosis could be challenging in the clinical scenario, and response to antimicrobial treatment could help to distinguish the initial trigger. The present would be among the first 15 cases to our knowledge describing the association between M. tuberculosis and alveolar proteinosis [ 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 ].…”

Pulmonary Alveolar Proteinosis Due to Pneumocystis carinii in Type 1 Hyper-IgM Syndrome: A Case Report

“…PAP is an intractable interstitial lung disease involving bilateral sedimentation of lipoprotein acetous material in the lungs, affecting gas exchange and impairing the pulmonary defense against pathogenic bacteria. The diagnosis of PAP is based on clinical symptoms, imaging data, characteristics of bronchoalveolar lavage fluid, and results of lung biopsy (12,13). The present case of PAP diagnosis was confirmed by evaluation of the bronchoalveolar lavage and pathology results.…”