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Febrile abdominal pain revealing Horton's disease
Arnaud Chaudet1, Jean-Michel Goujon2, Aiham Daniel Ghazali3
1Emergency Department, University Hospital of Poitiers, France.
Insights
Horton's disease, a rare cause of aortitis, can present with abdominal pain in the elderly. Early diagnosis and high-dose corticosteroid treatment are crucial for managing this serious vasculitis.
Area of Science:
- Rheumatology
- Vascular Medicine
- Internal Medicine
Background:
- Horton's disease (Giant Cell Arteritis) is a systemic inflammatory vasculitis affecting medium and large arteries, typically in individuals over 50.
- While commonly affecting cranial arteries like the superficial temporal artery, it can involve extracranial vessels, including the aorta, though this is rare.
- Aortitis, inflammation of the aorta, is challenging to diagnose and potentially underdiagnosed, especially when presenting without typical symptoms.
Observation:
- A 68-year-old woman presented with febrile abdominal pain and an inflammatory syndrome.
- Contrast-enhanced CT revealed aortitis extending from the descending aorta to the iliac arteries.
- Temporal artery biopsy confirmed Giant Cell Arteritis with intimal thickening and luminal stenosis.
Findings:
- The patient was diagnosed with Horton's disease based on age, temporal artery biopsy results, inflammatory syndrome, and associated aortitis.
- Aortitis, a rare but severe complication of Horton's disease, can lead to life-threatening conditions like aneurysm and dissection.
- This case highlights the unusual presentation of Horton's disease diagnosed via aortitis symptoms.
Implications:
- Horton's disease should be considered in the differential diagnosis of febrile abdominal pain in elderly patients, even without typical symptoms.
- Prompt initiation of high-dose corticosteroid therapy is essential for managing Horton's disease and its aortic complications.
- Early diagnosis and treatment of aortitis in Horton's disease are critical to prevent severe morbidity and mortality.
Background:
Horton's disease is a systemic inflammatory vasculitis, usually found in persons over 50years old. It affects medium and large-sized arteries stemming from the external carotid, especially the superficial temporal arteries. It can affect extracranial large vessels but only rarely the aorta. Diagnosis of aortitis is difficult and its incidence is probably underestimated.
Case Presentation:
A 68-year-old Caucasian woman consulted in an emergency department for febrile abdominal pain with inflammatory syndrome. Abdomen was soft with right-side flank sensitivity. A contrast-enhanced CT scan showed aortitis from the descending aorta to the iliac arteries without complication. Because of age, clinical presentation and aortitis, Horton disease was suspected. The temporal artery biopsy showed a histological aspect of degenerative endarteritis with intimal thickening and luminal stenosis. High-dose corticosteroid therapy was introduced which improved clinical conditions and resulted in the amendment of the pain.
Discussion:
In the present case, this patient had Horton's disease, based on 3 criteria of The American College of Rheumatology (age, temporal artery abnormalities and inflammatory syndrome) associated with aortitis. However, aortitis is a rare complication of Horton disease and is a major cause of mortality inasmuch as it can be complicated by aneurysm and dissection. It is unusual to diagnose Horton's disease from aortitis symptoms without complications. The aorta represents the most severe localization of Horton's disease. It should not be ignored in etiological hypotheses regarding febrile abdominal pain in the elderly. Corticosteroids should be started rapidly at high doses and temporal artery biopsy should be planned.
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