Takayasu Arteritis as a Secondary Cause of Arterial Hypertension

Abstract Takayasu arteritis (TA) is a rare type of vasculitis that affects mainly the aorta and its major branches. It is highly similar to giant cell arteritis (GCA), and differentiation between them may not be achieved even by histological examination. Arterial hypertension is typical of TA and is caused by stenosis of the renal arteries. Here we report the case of a 59-year-old woman, with a history of dyslipidemia and anemia, seen in the Internal Medicine department for resistant hypertension. Evaluation of secondary causes led to stenosis of the renal arteries. Assessment of target organ involvement was performed by computed tomography angiograph which revealed ectasia of the aortic arch and ascending aorta, tortuous course of the brachiocephalic trunk and the proximal portion of the right common carotid artery; positron-emission tomography which showed diffuse increased uptake in the ascending aorta, compatible with large vessels vasculitis. The patient was submitted to aortic valve replacement with a biological prosthesis combined with myocardial revascularization (Bentall-De Bono procedure). Aortic biopsy specimens showed anatomical and pathological features of GCA and TA. Due to persistently uncontrolled hypertension, prednisone 60 mg was initiated,with significant improvement in patient's condition.

Síndrome de platinea-ortodeoxia en una señora mayor: un desafío diagnóstico / Platypnea-orthodeoxia syndrome in an old lady – a clinical challenge

Introduction: Platypnea-orthodeoxia syndrome (POS) is a rare disordercharacterized by dyspnoea and oxygen desaturation in the upright position which improves in the supine position.Clinical case: 84 years old woman with hypertension and Parkinson disease. She came to the emergency department complainingof dyspnoea. At admission she was polypneic (35 bpm), spO2 80%,157/108mmHg, 115bpm, apyretic, normal pulmonary auscultation andwithout peripheral oedema. Oxygen supplementation was started withlack of response. The patient, which at first was lying in the bed withthe headboard at 45º, recovered the peripheral oxygen saturation to96-97% when the bed was placed at 0º. Diagnostic tests were performed, excluding cardiac myocardial infarction, pulmonary embolismand pneumonia. Transthoracic echocardiogram revealed an aneurysmatic interauricular septum with transoesophageal echocardiogramshowing an interauricular communication ostium secundum type witha septal aneurysm that led to a right to left shunt. The anatomical andclinical features did not allowed percutaneous or surgical closure. Aftera group discussion we decided for palliative care.Discussion: POS due to a septal aneurysm with right-to-left shunt is arare and difficult diagnose because it requires a detailed medical examination and exclusion of any other possible diseases. Besides that, itis a very rare presentation in such an old person. (AU)

Introducción: El síndrome de platipnea-ortodesoxia (SPO) es un trastorno poco común caracterizado por disnea y desaturación de oxígenoen posición erguida que mejora en posición supina.Caso clínico: mujer de 84 años con hipertensión y enfermedad de Parkinson. Acudió al servicio de urgencias quejándose de disnea. Al ingreso se encontraba taquipneica (35cpm), spO2 80%, 157/108mmHg,115bpm, apirética, auscultación pulmonar normal y sin edema periférico. La suplementación con oxígeno se inició con ausencia de respuesta. La paciente, que al principio estaba acostada en la cama con elcabecero a 45º, recuperó la saturación de oxígeno periférico al 96-97%cuando la cama se colocó a 0º. Se realizaron pruebas diagnósticas,excluyendo infarto de miocardio, embolia pulmonar y neumonía. Elecocardiograma transtorácico reveló un septo interauricular aneurismático con ecocardiograma transesofágico que mostraba comunicacióninterauricular tipo ostium secundum con aneurisma septal que conducíaa un shunt de derecha a izquierda. Las características anatómicas yclínicas no permitieron el cierre percutáneo o quirúrgico. Después deuna discusión en grupo, nos decidimos por los cuidados paliativos.Discusión: El SPO por aneurisma septal con derivación de derecha aizquierda es un diagnóstico raro y difícil porque requiere un examenmédico detallado y la exclusión de cualquier otra posible enfermedad. Además de eso, es una presentación muy rara en una personatan mayor. (AU)

IgG4-related disease with renal and orbital involvement: a clinical case.

INTRODUCTION: IgG4-related disease (IgG4-RD) is characterized by the growth of pseudotumors, which can affect almost every organ. Elevated serum IgG4 levels are present in only two-thirds of the patients and are not specific. Diagnosis is difficult and is usually based on a biopsy. CLINICAL CASE: A 39-year-old man presented complaints of low back pain and fever. A renal computed tomography (CT) scan revealed a voluminous mass next to the right kidney and a biopsy showed an inflammatory process and excluded a neoplasm. A follow-up CT scan at four months revealed total regression without any treatment. Three years later, the patient presented with diplopia and right proptosis. MRI of the orbits revealed a retro-ocular mass; biopsy excluded a malignant process. Infectious, autoimmune or paraneoplastic diseases were also excluded. Although the patient's IgG4 serum levels were normal, histopathological reobservation of the renal biopsy revealed IgG4-positive plasma cells, thus confirming the diagnosis. The patient was prescribed a daily dose of 40 mg of prednisolone and regression of the right orbital lesion was observed. DISCUSSION: IgG4-RD is a rare and recently described condition. Most anatomic pathology laboratories do not routinely test for it. Spontaneous pseudotumor remission is possible, even when associated with the renal phenotype, although this has not been described until now.