scholarly journals Pulmonary alveolar proteinosis with secondary Aspergillus infection: A case report

2021 ◽  
Vol 11 (3) ◽  
pp. 191-195
Author(s):  
Fanny Fachrucha ◽  
Sita Andarini ◽  
Mia Elhidsi ◽  
Rizky Yudha Irawan ◽  
Romi Beginta ◽  
...  
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Secondary Infection ◽  
Invasive Pulmonary Aspergillosis ◽  
Left Lung ◽  
Ground Glass Opacity ◽  
Lavage Fluid ◽  
Lung Lavage ◽  
High Resolution Computed Tomography ◽  
Alveolar Proteinosis ◽  
Macrophage Colony

Pulmonary alveolar proteinosis (PAP) is a rare disease with mostly due to autoimmune toward granulocyte-macrophage colony-stimulating factor. In some conditions, PAP followed with secondary infection. A 34-year-old woman came with progressive shortness of breath, chronic dry cough, and mild fever. The chest High-Resolution Computed Tomography showed ground-glass opacity with septal reticulation or known as the crazy-paving pattern, and a cavity on the upper lobe of the left lung. The patient underwent bronchoscopy for diagnostic and therapeutic measures and found milky appearance bronchoalveolar lavage fluid (BALF). The serum galactomannan came out positive. Fungal infection detected from the BALF culture, Aspergillus fumigatus, hence fulfilling the diagnosis of PAP with probable invasive pulmonary aspergillosis. The patient showed clinical improvement after undergoing whole lung lavage and given anti-fungal medications.

scholarly journals The Clinical Clues of Pulmonary Alveolar Proteinosis: A Report of 11 Cases and Literature Review

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Qiongya Mo ◽  
Bingbin Wang ◽  
Nian Dong ◽  
Lianmin Bao ◽  
Xiaoqiong Su ◽  
...  
Keyword(s):  
Bronchoalveolar Lavage Fluid ◽  
Clinical Symptoms ◽  
Pulmonary Alveolar Proteinosis ◽  
Relevant Literature ◽  
Neuron Specific Enolase ◽  
Lavage Fluid ◽  
Lung Lavage ◽  
Serum Markers ◽  
Open Lung Biopsy ◽  
Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease characterized by the abnormal alveolar accumulation of surfactant components. The diagnosis of PAP can be easily missed since it is rare and lacks specific clinical symptoms. It is of great importance to have a better understanding of the crucial clue to clinically diagnose PAP and take PAP into consideration in the differential diagnosis of interstitial pulmonary diseases or other diseases with similar manifestations. Here, we analyze the clinical characteristics of 11 cases of PAP patients in local hospital and review the relevant literature in order to provide more information in diagnosis and management of PAP. In our observation, cyfra21-1 and neuron-specific enolase (NSE) known as tumor markers probably can be useful serum markers for diagnosis of PAP. As for the method of pathologic diagnosis, open-lung biopsy was the gold standard but now it is less required because findings on examination of bronchoalveolar lavage fluid (BALF) can help to make the diagnosis. We also have deep experience about when and how to carry out lung lavage.

scholarly journals Pulmonary Alveolar Proteinosis (PAP) in pregnancy: An important spot diagnosis

2021 ◽  
Vol 5 (2) ◽  
pp. 16-18
Author(s):  
Choon Seong Ng ◽  
Boon Hau Ng
Keyword(s):  
Pulmonary Surfactant ◽  
Pulmonary Alveolar Proteinosis ◽  
Ground Glass Opacity ◽  
Lavage Fluid ◽  
Maternal Infection ◽  
Thoracic Imaging ◽  
Fetal Survival ◽  
Fluid Analysis ◽  
Alveolar Proteinosis ◽  
In Pregnancy

Pulmonary alveolar proteinosis is a relatively rare syndrome of pulmonary surfactant clearance dysfunction that could present like asthma. A middle-aged pregnant lady presented with asthma-like symptoms which was negative for autoimmune screening, whom thoracic imaging consistent with ground-glass opacity superimposed with septal thickening. Whole lung bronchopulmonary lavage fluid analysis showed predominantly eosiniphilic material within alveolar space. Subsequent lung biopsy revealed positive PAS stain for eosinophilic material. Its presentation in pregnancy could pose challenge to delivery. The associated maternal infection risk could compromise fetal survival.

scholarly journals Pulmonary alveolar proteinosis: a complete response to GM-CSF therapy

Thorax ◽  
2001 ◽  
Vol 56 (8) ◽  
pp. 664-665
Author(s):  
R M Barraclough ◽  
A J Gillies
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Rare Condition ◽  
Complete Response ◽  
Lung Lavage ◽  
Whole Lung Lavage ◽  
Gm Csf ◽  
Macrophage Colony Stimulating Factor ◽  
Alveolar Proteinosis ◽  
Macrophage Colony ◽  
New Treatment

Pulmonary alveolar proteinosis is a rare condition traditionally requiring treatment with whole lung lavage. The case is presented of a young man who obtained complete remission following treatment with granulocyte-macrophage colony stimulating factor, a new treatment option.

scholarly journals Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges

2012 ◽  
Vol 7 ◽  
Author(s):  
Ilaria Campo ◽  
Zamir Kadija ◽  
Francesca Mariani ◽  
Elena Paracchini ◽  
Giuseppe Rodi ◽  
...  
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Gene Mutations ◽  
Spontaneous Remission ◽  
Lung Lavage ◽  
Current Standard ◽  
Gm Csf ◽  
Genes Encoding ◽  
Current Standard Treatment ◽  
Alveolar Proteinosis ◽  
Macrophage Colony

Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals and in distinct clinical forms: autoimmune (previously referred to as the idiopathic form, represents the vast majority of PAP cases, and is associated with Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) auto-antibodies; GMAbs), secondary (is a consequence of underlying disorders), congenital (caused by mutations in the genes encoding for the GM-CSF receptor), and PAP-like syndromes (disorders associated with surfactant gene mutations). The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole lung lavage (WLL) is the current standard treatment for PAP patients and although it is effective in the majority of cases, disease persistence is not an unusual outcome, even if disease is well controlled by WLL. In this paper we review the therapeutic strategies which have been proposed for the treatment of PAP patients and the progress which has been made in the understanding of the disease pathogenesis.

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