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Colodetti R et al.
1028 Rev Assoc M ed Bras 2017; 63(12):1028-1031
IMAGING IN MEDICINE
Cogan’s syndrome – A rare aortitis, difficult to diagnose but with
therapeutic potential
Raiza Colodetti1, Guilherme Spina2, T atiana Leal3, Mucio Oliveira Jr4, Alexandre Soeiro3*
1MD Cardiologist, Instituto do Coração (InCor), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil
2Assistant Physician at the Valvular Heart Disease Outpatient Clinic, InCor, HC-FMUSP, São Paulo, SP, Brazil
3Assistant Physician at the Clinical Emergency Service, InCor, HC-FMUSP, São Paulo, SP, Brazil
4Director of the Clinical Emergency Service, InCor, HC-FMUSP, São Paulo, SP, Brazil
Summary
Study conducted at Unidade Clínica de
Emergência, Instituto do Coração (InCor),
Hospital das Clínicas, Faculdade de
Medicina da Universidade de São Paulo
(HC-FMUSP), São Paulo, SP, Brazil
Article received: 8/18/2017
Accepted for publication: 9/9/2017
*Correspondence:
Address: Av. Dr. Enéas de
Carvalho Aguiar, 44
São Paulo, SP – Brazil
Postal code: 05403-900
The inflammation of aortic wall, named aortitis, is a rare condition that can
be caused by a number of pathologies, mainly inflammatory or infectious in
nature. In this context, the occurrence of combined audiovestibular and/or
ocular manifestations eventually led to the diagnosis of Cogan’s syndrome,
making it the rare case, but susceptible to adequate immunosuppressive
treatment and satisfactory disease control.
Keywords: chest pain, aortitis, Cogan’s syndrome.
Introduction
Inflammation of the aortic wall, called aortitis, is an in-
frequent clinical condition that manifests itself with sys-
temic symptoms and may cause precordial pain.1-4 One
of the rheumatologic causes of aortitis is a rare disease
called Cogan’s syndrome.5 With approximately 300 cases
reported in the world, it has no clearly defined etiology,
pathophysiology, diagnosis or treatment,5 and affects
from children to the elderly but mostly young adults
(mean age 30 years).5 The occurrence of vestibular and
ophthalmologic symptoms associated with the condition
aided in the diagnosis of the case described, which, despite
being rare, may respond to adequate immunosuppressive
treatment achieving satisfactory disease control.6
Case report
This is the case of a 63 year-old male patient, Caucasian,
born and living in the city of São Paulo, who attended
the emergency department with a complaint of severe
precordial chest pain described as tightness and irradi-
ated to the mandible 2 hours before admission to hospi-
tal, combined with sweating and dyspnea. The patient
reported the repeated occurrence of the symptoms, al-
though less intense, for more than ten years, and that
four years ago the episodes began to intensify. He was
admitted to another service a week before for the same
reason, where he underwent coronary angiography, show-
ing no coronary obstruction, and an echocardiogram,
which revealed a slight dilatation of the ascending aorta.
In addition, the patient reported bilateral hypoacusis for
10 years (progressing to deafness in the right ear), rota-
tional vertigo for 8 years and hyperemia, pain and eye
tearing for two years. At admission, he did not regularly
use any medications. He denied having risk factors for
coronary heart disease or other comorbidities.
At physical examination, the patient presented a regular
general condition, he was sweating and pale, with a heart
rate of 75 beats per minute, breathing normally, with pe-
ripheral arterial oxygen saturation of 97%, blood pressure
of 130 x 80 mmHg in both arms, ictus not visible but pal-
pable with fingertip in the normal position at the fourth
intercostal space under the left midclavicular line, rhythmic
heart sounds, no bruit heard and pulmonary auscultation
with preserved vesicular murmur bilaterally without adven-
titious breath sounds. Pulse was heard bilaterally, wide and
symmetrical. Jugular stasis was absent bilaterally at 45 degrees.
ECG results revealed sinus rhythm, without abnormal-
ities suggestive of myocardial ischemia. At that moment, the