Horton disease, juvenile

Juvenile Horton Disease
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Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Headache, scalp tenderness, jaw claudication, visual disturbances
Complications	Vision loss, stroke
Onset	Typically in adolescence
Duration	Chronic
Types	N/A
Causes	Unknown, possibly autoimmune
Risks	Genetic predisposition, environmental factors
Diagnosis	Clinical evaluation, imaging, biopsy
Differential diagnosis	N/A
Prevention	N/A
Treatment	Corticosteroids, immunosuppressants
Medication	N/A
Prognosis	Variable, can lead to complications if untreated
Frequency	N/A
Deaths	N/A

Juvenile Horton Disease, also known as Juvenile Giant Cell Arteritis, is a rare form of vasculitis that primarily affects the large and medium-sized arteries, particularly the temporal artery. It is considered a variant of Giant Cell Arteritis (GCA), which is more commonly seen in adults over the age of 50. Juvenile Horton Disease is characterized by inflammation of the blood vessels, which can lead to a range of symptoms and complications.

Clinical Presentation

Patients with Juvenile Horton Disease typically present with symptoms similar to those seen in adult GCA, but the onset occurs in adolescence. Common symptoms include:

• Severe headache, often localized to the temples
• Scalp tenderness
• Jaw claudication, or pain in the jaw when chewing
• Visual disturbances, such as blurred vision or sudden vision loss

In some cases, systemic symptoms such as fever, fatigue, and weight loss may also be present.

Pathophysiology

The exact cause of Juvenile Horton Disease is unknown, but it is believed to be an autoimmune disease where the body's immune system mistakenly attacks the blood vessel walls. This leads to inflammation and the formation of giant cells, which are large, multinucleated cells that can be seen on biopsy.

Diagnosis

Diagnosis of Juvenile Horton Disease is based on clinical evaluation, laboratory tests, and imaging studies. A temporal artery biopsy is often performed to confirm the diagnosis, revealing the presence of giant cells and inflammation in the vessel wall.

Treatment

The mainstay of treatment for Juvenile Horton Disease is high-dose corticosteroids, such as prednisone, to reduce inflammation and prevent complications. In some cases, additional immunosuppressive medications may be used to help control the disease.

Prognosis

The prognosis for patients with Juvenile Horton Disease varies. With prompt and appropriate treatment, many patients can achieve remission and prevent serious complications. However, if left untreated, the disease can lead to significant complications, including permanent vision loss and increased risk of stroke.

Also see

• Giant Cell Arteritis
• Temporal Arteritis
• Vasculitis
• Autoimmune disease