Szatmári Viktor, Teske Erik, Nikkels Peter G J, Griese Matthias, de Jong Pim A, Grinwis Guy, Theegarten Dirk, Veraa Stefanie, van Steenbeek Frank G, Drent Marjolein, Bonella Francesco
Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University, Utrecht, The Netherlands.
Department of Pathology, University Medical Centre Utrecht, Utrecht, The Netherlands.
BMC Vet Res. 2015 Dec 9;11:302. doi: 10.1186/s12917-015-0613-4.
Pulmonary alveolar proteinosis is an extremely rare lung disease in animals and humans. It is characterized by the deposition of a large amount of phospholipoproteinaceous material in the alveoli. There are several possible etiologies, both congenital and acquired. Alveolar macrophages play an important role in the clearance of surfactant. This is the first report of pulmonary alveolar proteinosis in the feline species.
Pulmonary alveolar proteinosis was diagnosed in an 8-month-old cat with chronic tachypnea, failure to thrive and finally respiratory distress. The diagnosis was based on the milky appearance of the bronchoalveolar lavage fluid taken under general anesthesia after bronchoscopy. Because of the worsening respiratory distress and development of anorexia the kitten was euthanized. Histopathology of the lungs showed alveoli and bronchi filled with eosinophilic material. Electron microscopy revealed lamellated intra-alveolar bodies. As the granulocyte-macrophage colony-stimulating factor was elevated in the serum and no autoantibodies against granulocyte-macrophage colony-stimulating factor were detected, a primary hereditary pulmonary alveolar proteinosis was suspected. The underlying cause was thought to be a dysfunction of the receptor of the granulocyte-macrophage colony-stimulating factor, however, a mutation in the genes encoding the alpha and beta chains of this receptor has not been found.
This is the first description of pulmonary alveolar proteinosis in a cat. This kitten is thought to have a primary hereditary pulmonary alveolar proteinosis with a possible defect in the signalling pathway of the receptor of the granulocyte-macrophage colony-stimulating factor. The imaging and pathologic findings are similar to those of humans.
肺泡蛋白沉积症在动物和人类中是一种极其罕见的肺部疾病。其特征是大量磷脂蛋白物质在肺泡内沉积。病因有多种可能,包括先天性和后天性。肺泡巨噬细胞在表面活性物质的清除中起重要作用。这是猫科动物肺泡蛋白沉积症的首例报告。
一只8个月大的猫被诊断为肺泡蛋白沉积症,该猫患有慢性呼吸急促、发育不良,最终出现呼吸窘迫。诊断基于支气管镜检查后在全身麻醉下采集的支气管肺泡灌洗液呈乳状外观。由于呼吸窘迫加重和出现厌食,这只小猫被实施安乐死。肺部组织病理学显示肺泡和支气管充满嗜酸性物质。电子显微镜检查发现肺泡内有层状小体。由于血清中粒细胞巨噬细胞集落刺激因子升高,且未检测到抗粒细胞巨噬细胞集落刺激因子的自身抗体,怀疑为原发性遗传性肺泡蛋白沉积症。推测潜在病因是粒细胞巨噬细胞集落刺激因子受体功能障碍,然而,尚未发现编码该受体α链和β链的基因突变。
这是猫肺泡蛋白沉积症的首次描述。这只小猫被认为患有原发性遗传性肺泡蛋白沉积症,粒细胞巨噬细胞集落刺激因子受体的信号通路可能存在缺陷。影像学和病理学表现与人类相似。