Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome

doi:10.1016/0002-9343(86)90683-2

The American Journal of Medicine

Volume 80, Issue 6, June 1986, Pages 1187-1190

https://doi.org/10.1016/0002-9343(86)90683-2 Get rights and content

Abstract

This report describes two patients with acquired immune deficiency syndrome (AIDS) in whom respiratory failure and opportunistic infection associated with secondary alveolar proteinosis developed. In one patient, the alveolar proteinosis was apparently secondary to Mycobacterium tuberculosis and in the other to Pneumocystis carinii and cytomegalovirus infection. Both patients died of respiratory failure, and it was suspected that secondary alveolar proteinosis could have been a contributing cause of death.

References (8)

G Singh et al.
Pulmonary alveolar proteinosis: staining for surfactant apoprotein in alveolar proteinosis and in conditions simulating it
Chest
(1983)
Special Report: Epidemiologic aspects of the current outbreak of Kaposi's sarcoma and opportunistic infections
N Engl J Med
(1982)
A Steer
Focal pulmonary alveolar proteinosis in pulmonary tuberculosis
Arch Pathol
(1969)
JM Reyes et al.
Association of pulmonary alveolar lipoproteinosis with mycobacterial infection
Am J Clin Pathol
(1980)

There are more references available in the full text version of this article.

Cited by (77)

Pulmonary Alveolar Proteinosis Syndrome
2015, Murray and Nadel's Textbook of Respiratory Medicine: Volume 1,2, Sixth Edition
Pulmonary alveolar proteinosis and superinfection with pulmonary tuberculosis in a case
2012, Respiratory Medicine Case Reports
Citation Excerpt :
Very few cases were defined with pulmonary tuberculosis accompanied to PAP. Two of them as superinfection of the proteinosis in the adult patients with acquired immune deficiency syndrome, one case in a HIV positive infected child and one with the association proteinosis and diabetes mellitus.2–4,9,10 We think that the M. tuberculosis was evolved as a superinfection on PAP.
Pulmonary alveolar proteinosis (PAP) is a rare and diffuse lung process, characterized by the presence of alveolar spaces filled with amorphous eosinophilic material. Impaired macrophage function and impaired host defence due to abnormalities of surfactant proteins may favor the growth of microorganisms. The association of alveolar proteinosis with mycobacterial infections is rarely reported. The PAP and superinfection with pulmonary tuberculosis is defined by radiologic and histopathologic in a 46 year-old patient. The patients with PAP should be monitored for superinfection. It may cause the disease progression and radiological, clinical symptoms may improve with treatment of superinfection.
The molecular basis of pulmonary alveolar proteinosis
2010, Clinical Immunology
Pulmonary alveolar proteinosis (PAP) comprises a heterogenous group of diseases characterized by abnormal surfactant accumulation resulting in respiratory insufficiency, and defects in alveolar macrophage- and neutrophil-mediated host defense. Basic, clinical and translational research over the past two decades have raised PAP from obscurity, identifying the molecular pathogenesis in over 90% of cases as a spectrum of diseases involving the disruption of GM-CSF signaling. Autoimmune PAP represents the vast majority of cases and is caused by neutralizing GM-CSF autoantibodies. Genetic mutations that disrupt GM-CSF receptor signaling comprise a rare form of hereditary PAP. In both autoimmune and hereditary PAP, loss of GM-CSF signaling blocks the terminal differentiation of alveolar macrophages in the lungs impairing the ability of alveolar macrophages to catabolize surfactant and to perform many host defense functions. Secondary PAP occurs in a variety of clinical diseases that presumedly cause the syndrome by reducing the numbers or functions of alveolar macrophages, thereby impairing alveolar macrophage-mediated pulmonary surfactant clearance. A similar phenotype occurs in mice deficient in the production of GM-CSF or GM-CSF receptors. PAP and related research has uncovered a critical and emerging role for GM-CSF in the regulation of pulmonary surfactant homeostasis, lung host defense, and systemic immunity.
Pulmonary Manifestations of Hematologic and Oncologic Diseases
2009, Pulmonary Manifestations of Pediatric Diseases
Pulmonary alveolar proteinosis in the course of ICF type 2 syndrome
2023, Acta Haematologica Polonica
Autoimmune Pulmonary Alveolar Proteinosis
2022, American Journal of Respiratory and Critical Care Medicine

View all citing articles on Scopus

View full text

Case reportSecondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome

Abstract

Chest

Special Report: Epidemiologic aspects of the current outbreak of Kaposi's sarcoma and opportunistic infections

N Engl J Med

Focal pulmonary alveolar proteinosis in pulmonary tuberculosis

Arch Pathol

Association of pulmonary alveolar lipoproteinosis with mycobacterial infection

Am J Clin Pathol

Case report
Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome