Juvenile temporal arteritis

Other Names: Juvenile giant cell arteritis; JGCA; Juvenile cranial arteritis; Juvenile polymyalgia rheumatica; JPMR

Juvenile temporal arteritis is a rare form of vasculitis, a group of conditions that cause inflammation of the blood vessels. Unlike the classic form of temporal arteritis, this condition is generally diagnosed in late childhood or early adulthood and only affects the temporal arteries (located at the lower sides of the skull, directly underneath the temple)

Cause

The exact underlying cause of the condition is unknown.

Inheritance

It generally occurs sporadically in people with no family history of the condition.

Symptoms

Affected people often have no signs or symptoms aside from a painless nodule or lump in the temporal region. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

30%-79% of people have these symptoms

• Headache(Headaches)
• Skin nodule

5%-29% of people have these symptoms

• Allergic rhinitis(Hay fever)
• Conjunctivitis(Pink eye)
• Eosinophilia(High blood eosinophil count)

Diagnosis

To confirm a diagnosis, the patient will need a biopsy of a temporal artery (a minor procedure performed under local anaesthetic to remove a sample of one of the scalp arteries). However, up to 44% of patients will have a normal biopsy. Therefore it is difficult to know if a patient with a normal biopsy does or does not have GCA. Radiological examination of the temporal artery with ultrasound yields a halo sign. Contrast-enhanced brain MRI and CT is generally negative in this disorder. Recent studies have shown that 3T MRI using super high resolution imaging and contrast injection can non-invasively diagnose this disorder with high specificity and sensitivity.

Treatment

Juvenile temporal arteritis is often treated with surgical excision and rarely recurs. The U.S. Food and Drug Administration today expanded the approved use of subcutaneous Actemra (tocilizumab) to treat adults with giant cell arteritis. This new indication provides the first FDA-approved therapy, specific to this type of vasculitis.

NIH genetic and rare disease info

• NIH info on Juvenile temporal arteritis

Juvenile temporal arteritis is a rare disease.

Juvenile temporal arteritis Resources
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