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[Secondary pulmonary alveolar proteinosis in a transplant patient]
M Chevereau-Choquet1, S Marchand-Adam1, J Mankikian1
1Service de pneumologie, CHU, Tours, France.
Introduction:
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar accumulation of lipoproteinaceous material, caused by a macrophagic clearance disorder. We present a case of PAP in a patient taking the immunosuppressant drug mycophenolate mofetil (MMF) in the context of invasive pulmonary aspergillosis, of which we discuss the pathophysiology and treatment as reported in the literature.
Case Report:
A 43-year-old man with cardiomyopathy received a heart transplant and was treated by MMF, tacrolimus and corticosteroids. Three months after the transplant, he presented with acute oxygen-dependent respiratory failure. The diagnosis of PAP seemed likely on the CT scan and was confirmed by bronchoalveolar lavage, as was the diagnostic of invasive pulmonary aspergillosis (IPA). However, GM-CSF autoantibodies were not found. As there existed a suspicion of MMF imputability, the treatment was discontinued and an antifungal treatment was started. The patient was reassessed one month after discontinuation of MMF and found to have clinically and radiologically improved. Four other cases of MMF-induced PAP have been reported in the literature.
Conclusions:
MMF and IPA could be predisposing cofactors for the occurrence of secondary PAP.
Insights
Mycophenolate mofetil (MMF) may contribute to pulmonary alveolar proteinosis (PAP) in transplant patients, especially when invasive pulmonary aspergillosis (IPA) is present. Discontinuing MMF led to clinical improvement in a heart transplant recipient.
Area of Science:
- Pulmonology
- Immunosuppression Therapy
- Rare Diseases
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare lung disorder involving lipoprotein accumulation due to impaired macrophage function.
- This case report examines PAP in a heart transplant patient treated with immunosuppressants.
Observation:
- A 43-year-old heart transplant recipient developed acute respiratory failure three months post-transplant.
- The patient was on mycophenolate mofetil (MMF), tacrolimus, and corticosteroids. Invasive pulmonary aspergillosis (IPA) was also diagnosed.
- PAP was confirmed via bronchoalveolar lavage; MMF was suspected as a contributing factor.
Findings:
- Discontinuation of MMF resulted in significant clinical and radiological improvement.
- No GM-CSF autoantibodies were detected, suggesting an alternative mechanism for PAP development.
- Four prior cases of MMF-induced PAP were identified in the literature.
Implications:
- Mycophenolate mofetil (MMF) and invasive pulmonary aspergillosis (IPA) may act as cofactors in the development of secondary PAP.
- This highlights the importance of considering drug-induced PAP in immunosuppressed patients with respiratory symptoms.
- Further research into the pathophysiology of MMF-associated PAP is warranted.
