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Pulmonary alveolar proteinosis due to mycophenolate and cyclosporine combination therapy in a renal transplant recipient.

Hasan A, Ram R, Swamy T - Lung India (2014)

Bottom Line: Pulmonary alveolar proteinosis (PAP) is an orphan disease characterized by the accumulation of excess of surfactant within alveoli and bronchioles.Secondary PAP (S-PAP) can be induced by a host of inciting agents and is far more liable to progress to terminal respiratory failure.We describe a rare case of S-PAP occurring in a renal transplant recipient due to mycophenolate and cyclosporine combination-therapy, which resolved spontaneously following withdrawal of these drugs.

View Article: PubMed Central - PubMed

Affiliation: Department of Pulmonary Medicine, Owaisi Hospital and Research Centre, Banjara Hills, Hyderabad, India ; Department of Pulmonology, Care Hospitals, Banjara Hills, Hyderabad, India.

ABSTRACT
Pulmonary alveolar proteinosis (PAP) is an orphan disease characterized by the accumulation of excess of surfactant within alveoli and bronchioles. The primary form of PAP (P-PAP; also referred to as idiopathic or autoimmune) is the most common form. It is mediated through a circulating neutralizing antibody against granulocyte-macrophage colony-stimulating factor. Secondary PAP (S-PAP) can be induced by a host of inciting agents and is far more liable to progress to terminal respiratory failure. We describe a rare case of S-PAP occurring in a renal transplant recipient due to mycophenolate and cyclosporine combination-therapy, which resolved spontaneously following withdrawal of these drugs.

No MeSH data available.


Related in: MedlinePlus

(a) Transbronchial lung biopsies shows preserved lung architecture. Alveoli are filled with granular eosinophiic material that noticeably lacks inflammatory cells; (b) periodic acid-Schiff stained tissue after diastase treatment
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Figure 3: (a) Transbronchial lung biopsies shows preserved lung architecture. Alveoli are filled with granular eosinophiic material that noticeably lacks inflammatory cells; (b) periodic acid-Schiff stained tissue after diastase treatment

Mentions: Routine biochemistry was normal. Resting oxygen saturation was 86-88%, falling further on effort. The chest X-ray showed a bilateral perihilar and lower zone infiltrates [Figure 1]. A chest CT scan showed bilateral diffuse ground-glass haziness with superimposed interlobular septal thickening [Figure 2]. Serology for cytomegalovirus, human immunodeficiency virus, ANA, cytoplasmic antineutrophil cytoplasmic antibodies and perinuclear anti-neutrophil cytoplasmic antibodies was negative. Bronchoscopic alveolar lavage (BAL) revealed no gross inflammation. Silver methenamine stain and stains for acid fast bacteria (AFB) were negative. BAL fluid cultures (pyogenic and AFB) were negative. Transbronchial lung biopsies showed dilated alveoli [Figure 3a] filled with PAS-positive granular eosinophic material along with deeply eosinophilic structures; inflammatory cells were notably absent. The eosinophilic material was resistant to decolorization with diastase[4] [Figure 3b], which is conclusive of alveolar proteinosis. Anti GM-CSF antibodies are not useful in S-PAP.[1]


Pulmonary alveolar proteinosis due to mycophenolate and cyclosporine combination therapy in a renal transplant recipient.

Hasan A, Ram R, Swamy T - Lung India (2014)

(a) Transbronchial lung biopsies shows preserved lung architecture. Alveoli are filled with granular eosinophiic material that noticeably lacks inflammatory cells; (b) periodic acid-Schiff stained tissue after diastase treatment
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4129606&req=5

Figure 3: (a) Transbronchial lung biopsies shows preserved lung architecture. Alveoli are filled with granular eosinophiic material that noticeably lacks inflammatory cells; (b) periodic acid-Schiff stained tissue after diastase treatment
Mentions: Routine biochemistry was normal. Resting oxygen saturation was 86-88%, falling further on effort. The chest X-ray showed a bilateral perihilar and lower zone infiltrates [Figure 1]. A chest CT scan showed bilateral diffuse ground-glass haziness with superimposed interlobular septal thickening [Figure 2]. Serology for cytomegalovirus, human immunodeficiency virus, ANA, cytoplasmic antineutrophil cytoplasmic antibodies and perinuclear anti-neutrophil cytoplasmic antibodies was negative. Bronchoscopic alveolar lavage (BAL) revealed no gross inflammation. Silver methenamine stain and stains for acid fast bacteria (AFB) were negative. BAL fluid cultures (pyogenic and AFB) were negative. Transbronchial lung biopsies showed dilated alveoli [Figure 3a] filled with PAS-positive granular eosinophic material along with deeply eosinophilic structures; inflammatory cells were notably absent. The eosinophilic material was resistant to decolorization with diastase[4] [Figure 3b], which is conclusive of alveolar proteinosis. Anti GM-CSF antibodies are not useful in S-PAP.[1]

Bottom Line: Pulmonary alveolar proteinosis (PAP) is an orphan disease characterized by the accumulation of excess of surfactant within alveoli and bronchioles.Secondary PAP (S-PAP) can be induced by a host of inciting agents and is far more liable to progress to terminal respiratory failure.We describe a rare case of S-PAP occurring in a renal transplant recipient due to mycophenolate and cyclosporine combination-therapy, which resolved spontaneously following withdrawal of these drugs.

View Article: PubMed Central - PubMed

Affiliation: Department of Pulmonary Medicine, Owaisi Hospital and Research Centre, Banjara Hills, Hyderabad, India ; Department of Pulmonology, Care Hospitals, Banjara Hills, Hyderabad, India.

ABSTRACT
Pulmonary alveolar proteinosis (PAP) is an orphan disease characterized by the accumulation of excess of surfactant within alveoli and bronchioles. The primary form of PAP (P-PAP; also referred to as idiopathic or autoimmune) is the most common form. It is mediated through a circulating neutralizing antibody against granulocyte-macrophage colony-stimulating factor. Secondary PAP (S-PAP) can be induced by a host of inciting agents and is far more liable to progress to terminal respiratory failure. We describe a rare case of S-PAP occurring in a renal transplant recipient due to mycophenolate and cyclosporine combination-therapy, which resolved spontaneously following withdrawal of these drugs.

No MeSH data available.


Related in: MedlinePlus