Aortitis requiring aortic repair associated with glaucoma, thyroiditis, glaucoma, and neuropathy: case report
© Stöllberger et al; licensee BioMed Central Ltd. 2011
Received: 21 October 2010
Accepted: 16 May 2011
Published: 16 May 2011
Aortitis may be due to infectious and non-infectious causes. We observed aortitis, associated with glaucoma, thyroiditis, pericarditis, pleural effusion and neuropathy in a 63-years old woman. Despite antibiotic therapy, inflammatory signs persisted and resolved only after initiation of glucocorticoid therapy. Increasing aortic ectasia necessitated resection of the ascending aorta and implantation of a Vascutek 30 mm prosthesis. Histologically a granulomatous aortitis was diagnosed. Since all other possible causes were excluded, an immunological mechanism of the aortitis is suspected and possible triggering factors are discussed.
Aortitis of the ascending aorta may be due to infectious and non-infectious causes, comprising several systemic disorders [1, 2]. Aortitis, associated with glaucoma, thyroiditis, pericarditis, pleural effusion and neuropathy with prompt clinical response to glucocorticoids has not been described so far.
A 63-year old HIV-negative Iranian female was hospitalized in May 2004 because of fatigue, undulating fever, night sweats, and weight loss starting 8 weeks before. In 1991 and 1992 she had undergone bilateral iridectomy because of glaucoma. She has had 2 uneventful pregnancies and wan through menopause at the age of 52. Twenty-seven years ago, an intrauterine device (IUD) had been implanted but never been removed. She smoked 20 cigarettes/day.
Antibiotic therapy was stopped after 4 weeks, and 62.5 mg oral prednisolone was initiated. The patient's condition improved within a few days. The red cell blood count returned to normal values without transfusion of packed blood cells, the CRP levels and erythrocyte sedimentation rate nearly normalized. She was discharged with 47.5 mg metoprolol, 20 mg lisinopril, 25 mg hydrochlorothiazide, 4 mg doxazosin, 100 μg levothyroxine, 100 mg acetylsalicylic acid, 10 mg citalopram, 30 mg mirtazapine, 40 mg pantoprazol, 3 mg bromazepam and 25 mg prednisolone.
Non-infectious aortitis is a rare cause of aortic aneurysm. In one major study, it was detected in 9% of 513 patients with surgically resected aortic aneurysms, and more frequently in females than in males . Non-infectious aortitis may be due to Takayasu's disease, giant cell arteritis, spondylarthropathy, Behcet's syndrome, relapsing polychondritis, Cogan's syndrome, retroperitoneal fibrosis, ankylosing spondylitis, systemic lupus erythematodes, scleroderma, psoriasis, ulcerative colitis, Crohn's disease, radiation, Reiter's syndrome, or Wiskott-Aldrich syndrome [1, 2]. In our case however, all the above mentioned diseases were excluded clinically or by laboratory testing.
Though the patient responded well steroid therapy, an infectious aetiology of the disease could not completely be excluded. Infectious aortitis results from septic embolization to the vasa vasorum, hematogenous seeding of an existing aneurysm, or extension from a contiguous site of infection, especially in patients with atherosclerosis . Infectious aortitis may be due to Streptococcus pneumoniae, group A streptococci, Haemophilus inflenzae, Staphylococcus aureus, Salmonella species, Pseudomonas, Treponema pallidum, Mycobacterium tuberculosis, Capnocytophaga canimorsus or Pasteurella multicida[3–5]. However, the clinical presentation and laboratory findings did not indicate an infectious aetiology: Neither did she suffer from atherosclerosis nor was there any evidence of a contiguous site of infection.
It can be speculated that a potential site for a chronic infection or an immunologically mediated aortitis might be the IUD, which are known to increase the risk of pelvic inflammatory disease . Most pelvic inflammatory diseases, specially Chlamydia trachomatis infections are asymptomatic . The repeatedly positive serologic findings for Chlamydia sp. might indicate clinically silent pelvic inflammatory disease. Furthermore, IUDs are known to influence immune responses . Antibodies against smooth-muscle cells, which were repeatedly positive in our patient, are considered diagnostic markers for autoimmune hepatitis. However, they are also a frequent finding in the sera of apparently healthy females . It can be hypothesized, that the device within the uterus, an organ consisting of smooth muscle cells, might have stimulated antibody production. The benign clinical course during 5 years after aortic surgery, however, indicates that the pathogenic role of the IUD might not continue over a long time.
This case shows that in aortitis, despite extensive investigations, occasionally no definite aetiology can be found. Even if regression of symptoms and inflammatory signs occurs after initiation of corticosteroid therapy, follow-up is warranted because progression of aortic ectasia could indicate a need for vascular surgery.
"Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal."
List of abbreviations
Human immunodeficiency virus
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