A Rare Occurrence of Pulmonary Alveolar Proteinosis after Lung Transplantation

Abstract

We present a case of pulmonary alveolar proteinosis (PAP) initially diagnosed 28 months after left single-lung transplantation for idiopathic pulmonary fibrosis. The diagnosis was based upon the presence of periodic acid-Schiff (PAS)-positive and surfactant immunostain–positive acellular lipoproteinaceous material within alveoli seen on transbronchial biopsy as well as in bronchoalveolar lavage fluid. The patient eventually also displayed a characteristic “crazy paving” pattern on radiographic imaging. Granulocyte macrophage-colony stimulating factor antibodies were negative, consistent with secondary PAP. PAP is a rare interstitial lung disease with only a few reported cases occurring after lung transplantation. The etiology is thought to be related to a defect in macrophage function caused by immunosuppression. Reduced immunosuppression has been associated with stabilization, but not reversal, of the condition in the case reported here. PAP is an exceptionally rare cause of dyspnea and radiographic infiltrates after lung transplantation and may be related to toxicity of immune-suppressive medications.

Keywords

lung transplantation - pulmonary alveolar proteinosis - interstitial lung disease - immunosuppression

• References

• 1 Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med 1958; 258 (23) 1123-1142
• 2 Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med 2003; 349 (26) 2527-2539
• 3 Wang T, Lazar CA, Fishbein MC, Lynch III JP. Pulmonary alveolar proteinosis. Semin Respir Crit Care Med 2012; 33 (5) 498-508
• 4 Christie JD, Edwards LB, Kucheryavaya AY , et al; International Society of Heart and Lung Transplantation. The Registry of the International Society for Heart and Lung Transplantation: 29th adult lung and heart-lung transplant report-2012. J Heart Lung Transplant 2012; 31 (10) 1073-1086
• 5 Yousem SA. Alveolar lipoproteinosis in lung allograft recipients. Hum Pathol 1997; 28 (12) 1383-1386
• 6 Parker LA, Novotny DB. Recurrent alveolar proteinosis following double lung transplantation. Chest 1997; 111 (5) 1457-1458
• 7 Bag R, Cagle PT, Allen T, Abrams J, Frost AE. De novo alveolar proteinosis in lung transplant recipients [abstract]. J Heart Lung Transplant 2003; 22 (1, Supplement): S139
• 8 Gal AA, Bryan JA, Kanter KR, Lawrence EC. Cytopathology of pulmonary alveolar proteinosis complicating lung transplantation. J Heart Lung Transplant 2004; 23 (1) 135-138
• 9 Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med 2002; 166 (2) 215-235
• 10 Shah PL, Hansell D, Lawson PR, Reid KB, Morgan C. Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis. Thorax 2000; 55 (1) 67-77
• 11 Carey B, Trapnell BC. The molecular basis of pulmonary alveolar proteinosis. Clin Immunol 2010; 135 (2) 223-235
• 12 Kitamura T, Tanaka N, Watanabe J , et al. Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor. J Exp Med 1999; 190 (6) 875-880
• 13 Bonfield TL, Russell D, Burgess S, Malur A, Kavuru MS, Thomassen MJ. Autoantibodies against granulocyte macrophage colony-stimulating factor are diagnostic for pulmonary alveolar proteinosis. Am J Respir Cell Mol Biol 2002; 27 (4) 481-486
• 14 Bedrossian CW, Luna MA, Conklin RH, Miller WC. Alveolar proteinosis as a consequence of immunosuppression. A hypothesis based on clinical and pathologic observations. Hum Pathol 1980; 11 (5, Suppl): 527-535
• 15 Kadikoy H, Paolini M, Achkar K , et al. Pulmonary alveolar proteinosis in a kidney transplant: a rare complication of sirolimus. Nephrol Dial Transplant 2010; 25 (8) 2795-2798
• 16 Chen CM, Ikegami M, Ueda T, Polk DH, Jobe AH. Exogenous surfactant function in very preterm lambs with and without fetal corticosteroid treatment. J Appl Physiol 1995; 78 (3) 955-960
• 17 Ioachimescu OC, Kavuru MS. Pulmonary alveolar proteinosis. Chron Respir Dis 2006; 3 (3) 149-159
• 18 Luisetti M, Kadija Z, Mariani F, Rodi G, Campo I, Trapnell BC. Therapy options in pulmonary alveolar proteinosis. Ther Adv Respir Dis 2010; 4 (4) 239-248
• 19 Huizar I, Kavuru MS. Alveolar proteinosis syndrome: pathogenesis, diagnosis, and management. Curr Opin Pulm Med 2009; 15 (5) 491-498
• 20 Kavuru MS, Sullivan EJ, Piccin R, Thomassen MJ, Stoller JK. Exogenous granulocyte-macrophage colony-stimulating factor administration for pulmonary alveolar proteinosis. Am J Respir Crit Care Med 2000; 161 (4 Pt 1) 1143-1148
• 21 Seymour JF, Dunn AR, Vincent JM, Presneill JJ, Pain MC. Efficacy of granulocyte-macrophage colony-stimulating factor in acquired alveolar proteinosis. N Engl J Med 1996; 335 (25) 1924-1925
• 22 Cordonnier C, Fleury-Feith J, Escudier E, Atassi K, Bernaudin JF. Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients. Am J Respir Crit Care Med 1994; 149 (3 Pt 1) 788-794
• 23 Menard KJ. Whole lung lavage in the treatment of pulmonary alveolar proteinosis. J Perianesth Nurs 2005; 20 (2) 114-126
• 24 Sato M, Hwang DM, Ohmori-Matsuda K , et al. Revisiting the pathologic finding of diffuse alveolar damage after lung transplantation. J Heart Lung Transplant 2012; 31 (4) 354-363
• 25 Ho VT, Mirza NQ, Junco Dd Dd, Okamura T, Przepiorka D. The effect of hematopoietic growth factors on the risk of graft-vs-host disease after allogeneic hematopoietic stem cell transplantation: a meta-analysis. Bone Marrow Transplant 2003; 32 (8) 771-775