Vasculitis of the middle vessels

Peri Arteritis Nodosa or PAN

Periarteritis nodosa (PAN) is a very rare necrotizing angeitis that can affect many organs, the cause of which is not well known (some forms are believed to be linked to the hepatitis B virus).

Patients often have a deterioration in their general condition with weight loss, fever, etc.

Muscle pain is present in half of the cases. They are intense, diffuse, spontaneous or triggered by pressure, which can nail the patient to bed due to the intensity of the pain and muscle wasting …

Joint pain predominates in large peripheral joints: knees, ankles, elbows and wrists.

Damage to the nerves called multineuritis is often seen, affecting several nerves such as sciatica, the external or internal popliteal, radial, ulnar or median nerve and is often associated with distal segmental edema. Untreated neuritis eventually leads to atrophy of the muscles innervated by the affected nerve.

Vasculitis can also affect the brain more rarely, which can lead to epilepsy, hemiplegia, stroke, ischemia or hemorrhage.

The suggestive sign on the cutaneous level is the purpura (purplish spots that do not fade when pressed) bulging and infiltrated, especially in the lower limbs or a livedo, forming kinds of meshes (livedo reticularis) or mottles (livedo racemosa) purplish on the legs. We can also see Raynaud’s phenomenon (a few fingers turn white in the cold), or even finger or toe gangrene.

Orchitis (inflammation of a testicle) is one of the most typical manifestations of PAN, caused by vasculitis of the testicular artery which can lead to testicular necrosis.

A biological inflammatory syndrome is present in the majority of patients with PAN (increase in sedimentation rate to more than 60 mm in the first hour, in C Reactive Protein, etc.), major hyper eosinophilia (increase in eosinophilic polynuclear white blood cells ).

Hepatitis B infection results in the presence of HBs antigen in approximately ¼ to 1/3 of patients

Angiography reveals microaneurysms and stenosis (decrease in caliber or tapering appearance) of medium caliber vessels.

Treatment of PAN begins with corticosteroid therapy, sometimes combined with immunosuppressants (especially cyclophosphamide)

Biotherapies take place in the management of PAN, in particular rituximab (anti-CD20).

Buerger disease

Buerger’s disease or thromboangiitis obliterans is an angiitis affecting segments of small and medium arteries and veins of the lower and upper limbs, causing thrombosis and recanalization of the affected vessels. This disease is more common in Asia and among Ashkenazi Jews.

It occurs in a young patient (less than 45 years old), often smoker, who begins to present manifestations of arteritis early in life (ischemia of the fingers or toes, intermittent claudication, ischemic arterial ulcers or gangrene of the legs, etc.)

Arteriography reveals damage to the distal arteries.

Treatment involves stopping smoking altogether, which is a trigger and aggravation of the disease.

The doctor prescribes vasodilators and antiplatelet drugs such as aspirin

Revascularization surgery may be required.

Kawasaki’s Maladie

Kawasaki disease or “adeno-cutaneous-mucous syndrome” is a vasculitis electively affecting the territory of the coronary arteries responsible in particular for coronary aneurysms which can be a source of mortality, especially in children between 6 months and 5 years old with a peak frequency at the age of 18 months.

The disease takes place in three phases over several weeks

Acute phase (lasting 7 to 14 days): fever with a rash and appearance of “cherry lips”, “strawberry tongue”, “eyes injected” by bilateral conjunctivitis, “inconsolable child”, edema and redness of the hands and feet. Ideally, treatment should be started at this stage to limit the risk of cardiac sequelae

Subacute phase (14 to 28 days) resulting in peeling of the pulp of the fingers and toes beginning around the nails. It is at this stage that coronary aneurysms form

Convalescent phase, usually symptom-free, but during which sudden cardiac complications can occur due to the formation of coronary aneurysms in the previous phase.

The other signs are diaper rash, bright red with a desquamative ruff, cardiovascular signs (heart murmur, cardiac gallop, Electro CardioGram abnormalities, pericarditis, myocarditis …), digestive (diarrhea, vomiting, abdominal pain …), Neurological (aseptic meningitis, convulsions, paralysis), urinary (sterile pus in the urine, urethritis), polyarthritis…

Significant inflammation in the blood is demonstrated with a Sedimentation Rate greater than 100mm in the first hour and a very high C-reactive protein, a marked increase in polynuclear white blood cells greater than 20 elements / mm000, and an increase in platelets.

Treatment is based on immunoglobulins injected intravenously (IV Ig) as early as possible to limit the risk of coronary aneurysm. If IVIG is not effective, doctors use intravenous cortisone or aspirin.