Renal Artery Dissection in a Young Woman: Diagnoses Beyond Fibromuscular Dysplasia
NEPHRON
Authors: Sanchez-Baya, M.; Gali, A. Ramos; Barros-Membrilla, A.; Guerrero, R.; Villalba, J.; Martinez, M. J.; Moya, P.; Calero, F.; Guirado, L.; Fernandez-Llama, Patricia
Abstract
A 31-year-old woman presented at the emergency room after experiencing colic pain in the right iliac fossa for 5 days. She had previously consulted another center, where deterioration of renal function had been identified and abdominal computed tomography (CT) angiography had shown a dissection of the right renal artery, with areas suggestive of infarction in the right kidney, as well as an aneurysm in the left renal artery and a smaller left kidney. The patient had no relevant family or personal history except posttraumatic carotid-cavernous fistula in 2014, which had been treated with embolization. In our hospital, the patient was hypertensive and acute renal failure was confirmed, accompanied by an increase in lactate dehydrogenase and isomorphic microhematuria. After a new CT Scan, in addition to the lesions described in the renal arteries, another aneurysm in the splenic artery and an aneurysm of the right femoral artery were identified. Antihypertensive treatment was initiated with calcium antagonists and anticoagulation. Subsequent renal arteriography confirmed the dissection of the right renal artery, which could not be repaired, and a coated stent was placed in the left renal artery to exclude the aneurysm. The splenic artery lesion was treated 2 months later. The etiological diagnosis in this young woman was challenging. The presence of visceral aneurysms suggested a differential diagnosis comprising fibromuscular dysplasia, vasculitis, and collagenopathies. Using a multidisciplinary approach and directed anamnesis, the presence of frequent sprains, joint hypermobility, and skin fragility was confirmed. Blood immunology and CT angiography including the thoracic and cervical territories were normal. Echocardiography revealed tricuspid insufficiency. All these data suggested the presence of a collagen-like Ehlers-Danlos syndrome (vascular form). The diagnosis was confirmed by the genetic study, which showed a pathogenic mutation in the COL3A1 gene. Currently, the patient is asymptomatic with recovered renal function following treatment with a beta-blocker and antiplatelet therapy.
RELM alpha Is Induced in Airway Epithelial Cells by Oncostatin M without Requirement of STAT6 or IL-6 in Mouse Lungs In Vivo
CELLS
Authors: Ho, Lilian; Yip, Ashley; Lao, Francis; Botelho, Fernando; Richards, Carl D.
Abstract
Resistin-like molecule alpha (RELM alpha) and YM-1 are secreted proteins implicated in murine models of alternatively activated macrophage (AA/M2) accumulation and Th2-skewed inflammation. Since the gp130 cytokine Oncostatin M (OSM) induces a Th2-like cytokine and AA/M2 skewed inflammation in mouse lung, we here investigated regulation of RELM alpha and YM-1. Transient pulmonary overexpression of OSM by Adenovirus vector (AdOSM) markedly induced RELM alpha and YM-1 protein expression in total lung. In situ hybridization showed that RELM alpha mRNA was highly induced in airway epithelial cells (AEC) and was co-expressed with CD68 mRNA in some but not all CD68+ cells in parenchyma. IL-6 overexpression (a comparator gp130 cytokine) induced RELM alpha, but at significantly lower levels. IL-6 (assessing IL-6(-/-)mice) was not required, nor was STAT6 (IL-4/13 canonical signalling) for AdOSM-induction of RELM alpha in AEC. AEC responded directly to OSM in vitro as assessed by pSTAT3 activation. RELM alpha-deficient mice showed similar inflammatory cell infiltration and cytokine responses to wt in response to AdOSM, but showed less accumulation of CD206+ AA/M2 macrophages, reduced induction of extracellular matrix gene mRNAs for COL1A1, COL3A1, MMP13, and TIMP1, and reduced parenchymal alpha smooth muscle actin. Thus, RELM alpha is regulated by OSM in AEC and contributes to extracellular matrix remodelling in mouse lung.