From new treatments to the complex patient journey, 2024 brought pivotal insights into aPAP
Key studies and patient experiences highlight breakthrough treatments, diagnostic challenges, and the impact of fibrosis in this rare lung disease.
1. Rare case of pulmonary alveolar proteinosis linked to Bacillus megaterium infection
Pulmonary alveolar proteinosis, commonly linked to autoimmune disorders, can also result from rare infections like Bacillus megaterium, as demonstrated in a recent case study. A patient with a persistent cough and dyspnea was diagnosed after imaging showed a “crazy paving” pattern and a CT-guided biopsy confirmed B. megaterium infection, with subsequent antimicrobial treatment leading to significant clinical improvement.
Read more here.
2. Often misdiagnosed, patients face a complicated journey with autoimmune pulmonary alveolar proteinosis
Swaminathan Perinkulam Sathyanarayanan, MBBS, a second-year fellow in pulmonary and critical care medicine at the University of Florida in Gainesville, discusses an analysis presented at the 2024 CHEST Annual Meeting that highlighted the patient journey with autoimmune pulmonary alveolar proteinosis.
Watch here.
3. Pulmonary fibrosis affects 1 in 4 patients with autoimmune PAP, study finds
A study on autoimmune pulmonary alveolar proteinosis found that about 25% of patients develop pulmonary fibrosis over time, with dust exposure significantly increasing the risk. Despite treatments like whole-lung lavage and rituximab, fibrosis was associated with worse outcomes, including higher mortality rates.
Read more here.
4. Transdermal Semen Strychni shows promise in treating aPAP
Transdermal Semen Strychni (SS) therapy has been shown to be a safe and effective treatment for autoimmune pulmonary alveolar proteinosis, with significant improvements in gas diffusion metrics and lipid profiles compared to a placebo. In a randomized controlled trial, patients treated with SS showed reduced A-aDo2, increased DLCO % predicted, and lowered cholesterol levels, with no serious adverse events reported.
Read more here.
5. Patient journey map for aPAP presented at CHEST 2024
A poster at the 2024 CHEST Annual Meeting presented a Patient Journey Map (PJM) for autoimmune pulmonary alveolar proteinosis, emphasizing the complex and often frustrating diagnostic and treatment processes, as well as the emotional and financial burdens on patients. The PJM revealed inconsistent diagnoses, lengthy delays, misdiagnoses, and significant barriers to care, with patients calling for standardized guidelines, better education for healthcare providers, and improved access to specialists.
Read more here.
