The vascularities: what is it?

The vascularities: what is it?

Vasculitis are inflammations of the walls of the vessels leading to an alteration of the vascular wall. They are also called vasculitis or angeitis.

A distinction is made between large, medium and small vasculitis:

  • great vessels: aorta and its branches
  • small vessels: capillaries
  • middle vessels: intermediate vessels

Vasculitis of the great vessels

Horton’s disease

Horton’s disease is of unknown cause, affecting exclusively people over 60 years of age, particularly in the external carotid network: it is therefore responsible for many ischemic phenomena in the face, including an anomaly of the temporal arteries, mandibular claudication. , necrosis of the face (tongue and lips) or scalp, headaches, pain and hypersensitivity of the scalp. These are the ophthalmologic signs which can be serious (loss of visual acuity, oculomotor paralysis, ptosis, pupillary anomaly) and lead to blindness by ischemic optic neuritis generally irreversible.

The preceding signs may be missing or be associated with an alteration of the general condition, fever, vascular anomalies (limb limb, inflammatory arterial aneurysms, especially aortic, or even certain forms of cerebrovascular accidents, myocardial ischemia or digestive, Raynaud’s syndrome), rheumatological (pseudo-rhizomelic arthritis) or respiratory (unexplained dry cough).

The clinical signs are often not very evocative, the attention is generally drawn by a very important inflammatory syndrome (speed of sedimentation greater than 80 mm at the 1st hour) and disruption of the proteins of inflammation in the blood.

Examination of the temporal arteries by arterial color-Doppler ultrasound shows arterial flow abnormalities associated with a peri-arterial inflammatory halo.

The diagnosis is confirmed by pathological examination under a microscope of an arterial fragment, most often the superficial temporal artery with a size of at least 3 cm.

Oral corticosteroid therapy is the standard treatment for Horton’s disease.

Takayasu’s disease

Takayasu’s disease or “disease of women without a pulse” is of unknown origin, affecting the aorta, its branches (coronary arteries, carotids, subclavians, vertebral, renal, iliac) as well as the pulmonary arteries of hence its name of “aortic arch syndrome”.

This rare disease mainly affects young women between 20 and 35 years old. It is found more frequently in Asia.

We distinguish the acute period, known as pre-occlusive and the occlusive period.

La pre-occlusive period is characterized by the appearance of general signs that are not very specific (fever, joint pain, deterioration in general condition, weight loss, etc.).

Sometimes there is pain in the eye, visual disturbances, erythema nodosum

Then appear neck pain along the path of the carotid artery (carotinodynia) more suggestive of the disease, pain in the upper limbs, weakness or more pronounced fatigability from one side to the other.

Now occlusive period, the arteries become narrowed (stenosis) or occluded. We can realize this fortuitously when taking the different blood pressure between the 2 arms or an absence of pulse in places.

The arterial color Doppler ultrasound, the angio-scanner, or the angio-MRI, the PET-scanner (positron emission tomography) and the arteriography show stenosis in “candle flame” or “tail. radish ”, arterial obstructions or aneurysms, inflammation of the wall of the aorta (aortitis).

Treatment is based on corticosteroid therapy for one to two years, sometimes combined with immunosuppressive therapy (methotrexate, azathioprine, mycophenolate mofetil).

In case of resistance to treatment, biotherapies may be tried: anti-TNF (Tumor Necrosis Factor) (infliximab) or anti-interleukin 6 receptors (tocilizumab)

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