Pulmonary Alveolar Proteinosis- A Case Report

Abstract

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.