Horton’s disease

Horton’s disease

N BELHADJ

Associate Professor of Internal Medicine. 

1. Definition

Horton’s disease is characterized by inflammation of the  artery walls . It particularly affects the temporal arteries, located on the temples at the level of the hairline, which is why it is also called  arteritis.  or giant cell arteritis.

Horton’s disease mainly affects people over 50 years of age, with a peak in frequency around 75-80 years of age.

2. Clinical signs

Fatigue, weight loss

Fever

Severe headaches that spread to the temples and neck

Difficulty chewing (jaw claudication)

Swelling of the temporal arteries and absence of pulse Joint pain and stiffness  in the  pelvis , shoulders and neck (pseudo-polyarthritis rhizomelic)

Raynaud’s syndrome 

Dermatological and respiratory symptoms (dry cough)

Specific signs of temporal artery syndrome:

• Temporal artery hardened
• Absence of temporal pulse, redness in relation to the artery
• Lockjaw
• Intermittent jaw claudication: pain when chewing
• Hyperesthesia of the scalp
• Dysgeusia
• Tongue necrosis
• Localized scalp necrosis

3. Clinical and biological manifestation linked to the inflammation syndrome:

• Asthenia, fever, weight loss may be the only signs in 10% of patients
• Asthenia is common
• Fever is observed in more than half of the cases, most often moderate around 38°C.
• Weight loss is often associated with anorexia, and is usually moderate, but can sometimes be greater than 10% of body weight.
• The ESR is greater than 50 mm in the first hour and a normal ESR is found in only 2% of cases.
• CRP is among the proteins of inflammation considered as the best marker
• Moderate inflammatory anemia is observed in 50 to 80% of cases.
• Neutrophilic hyperleukocytosis is common. Hyperplaquettosis is common.
• Apart from the elevation of platelets and fibrinogen, an activation of coagulation in the active phase of Horton’s disease is discussed.

Note: Any unexplained headache pain that has recently appeared and persists in an elderly person requires an inflammatory assessment. 

• The main complication is vision loss (amaurosis), with a sudden, unilateral drop in visual acuity . . 
• 10% of patients are thus affected by total and irreversible blindness.

4. Temporal artery biopsy (TAB):

• Although clinical elements alone allow the diagnosis to be made, anatomo-pathological examination is always desirable.
• The biopsy, which almost exclusively concerns the superficial temporal artery or its anterior branch, is a simple surgical procedure performed under local anesthesia, but must be carried out rigorously (1 cm of the artery).
• It shows an inflammatory arteritis lesion with granulomatous infiltration (giant cells) predominant in the media leading to a rupture of the internal elastic lamina.
• BAT is positive in only 70% of cases, 
• After 15 days of corticosteroid therapy, sensitivity collapses.

5. Causes of Horton’s disease

Horton’s disease is caused by the infiltration of immune cells (giant cells) into the walls of the arteries.

 The latter thickens and swells, which prevents the passage of blood and causes a lack of oxygenation.

 It is this poor oxygenation that leads to headaches and degeneration of the optic nerve (   acute anterior  ischemic optic neuritis, AION).

The exact causes of the disease are unknown, and it may be due to a disorder of the  immune system  that turns against the body (autoimmune disease).

 Genetic factors are also involved.

• The highly varied clinical manifestations of GCA, including atypical orofacial pain, represent a challenge for the dentist, who may be the first practitioner the patient consults and who must be able to exclude dental causes, suspect GCA in the presence of any recent headache pain in a patient over 50 years of age, and refer the patient quickly. 

Exp: 

• A 63-year-old female patient was referred for consultation to the dental department by her treating dentist for advice regarding tooth 17. 
• The patient’s history revealed:

Shooting and throbbing pains that began one month previously in the posterior cervical and occipital region, radiating to the right side to the ear, maxilla, buccal vestibule and upper gum

• The clinical and radiological oral examination carried out by the practitioner revealed pain on percussion of the 17th, pulpless and crowned, and an apical radiolucent image opposite the palatal root. 
• The diagnosis of periradicular inflammatory lesion of endodontic origin was made; antibiotic therapy with amoxicillin 2 g/day and metronidazole 1.5 g/day was prescribed for 7 days. 
• The persistence of the pain raised suspicion of a fracture or root crack of the tooth and the patient was referred for specialist advice and hospital care. The endodontic consultation clinically demonstrated low-intensity pain on percussion of tooth 17, a negative bite test and the absence of fistula, swelling or periodontal pocket at the level of this tooth. 
• She was referred the same day for an oral surgery consultation for suspected Arnold’s neuralgia (due to occipital and cervical pain) or trigeminal neuralgia (due to maxillary pain). During this consultation, the clinical interview was repeated. In addition to the initial pain, it revealed new symptoms: temporal headache, asthenia, anorexia and intermittent visual disturbances such as blurred vision. 
• Clinical examination of the cephalic region revealed the presence of a prominent, indurated right temporal artery covered by inflammatory-appearing skin and the disappearance of the right temporal pulse. 
• The diagnosis of ACG was suggested and the patient was referred urgently for a rheumatology consultation.
• In the presence of a significant biological inflammatory syndrome (erythrocyte sedimentation rate, ESR = 56 mm/1 h, and C-reactive protein, CRP = 96 mg/l), a biopsy of the right temporal artery was performed. 
• Atypical orofacial manifestations of GCA may be misinterpreted by the practitioners the patient first consults and lead to a diagnostic delay, responsible for permanent blindness or a stroke, in the absence of rapid and appropriate treatment. Thus, dentists must know how to include GCA in the differential diagnoses of oro-cervicofacial pain. 
• Treatments are based on corticosteroid therapy and long-term immunosuppressants and anticoagulant or antiplatelet therapy as well as possible anti-bone resorption treatment requiring special precautions during oral and dental surgical procedures that patients with GCA may subsequently require. 

Behcet’s disease

1. Definition

• Behçet’s disease is   a  systemic vasculitis  , a disease related to inflammation of  the   body’s blood vessels .
• It occurs mainly in young adults, and most often manifests itself through cutaneous-mucosal symptoms ( aphthosis ), articular (pain) and ophthalmic ( uveitis ). 
• More rarely, lesions can affect the nervous system ( meningitis , cerebral venous thrombosis), digestive system (pain, bleeding) and cardiovascular system (thrombosis,  aneurysm ).

2. Clinical signs:
3. Skin signs

Mouth ulcers: ulcers with clear erythematous (red) edges, raised (1-3 cm in diameter), painful, isolated or in groups (clusters), developing in flare-ups of varying intensity 

 This involvement is almost constant in the disease ^. The recurrent nature is an indication in favor of Behçet’s 

Genital ulcers, inconstant (in 50% to 80% of cases), very suggestive of Behçet’s, which can leave scars that must be interpreted; the same goes for rare scrotal conditions. 

Cutaneous pseudofolliculitis  : these are common (60% of cases). They are located on the back, face, lower limbs, buttocks and scrotum. These are papules that become covered in two or three days with a vesicle that becomes a pustule, then a crust that comes off without leaving a trace.

The contents of the vesicles are sterile. This lesion is not centered by a hair.

Hyper-reactivity to trauma, or even authentic  erythema nodosum  in just under one in two cases. 

2. Eye signs

Anterior uveitis with  hypopyon  is  characteristic. Posterior uveitis  is more hidden, but sometimes has a severe prognosis. 

3. Vascular signs: vasculitis

It is essentially  thrombosis  of large vessels (veins or arteries) occurring due to inflammation of their wall.

Venous involvement with superficial, deep, or visceral thrombophlebitis.

Arterial damage with thrombosis, aneurysms, of any artery.

4. Joint signs

Predilection for large joints of the lower limbs, particularly the knees and hips.

Acute mono or oligoarthritis 

chronic monoarthritis 

More or less symmetrical bilateral polyarthritis simulating rheumatoid arthritis 

5. Neurological signs

Headaches

Meningoencephalitis.

Psychiatric disorders

6. Gastrointestinal signs

Aphthous or ulcerative lesions may affect the gastrointestinal tract, especially the ileocecal junction and ascending colon, potentially leading to hemorrhage and perforation.

7. Respiratory signs

Pulmonary infiltrates and pleurisy are possible. 

Possibilities to rule out are pulmonary embolism and pulmonary artery vasculitis, or even pulmonary artery aneurysms.

8. Renal signs

A high level of protein in the urine, associated with phenomena of urinary lithiasis (urinary stones causing attacks of renal colic). 

Subacute glomerulopathy remains rare.

3. Additional examinations:

Biology shows hyperleukocytosis with a predominance of polymorphonuclear neutrophils (very non-specific).

There is a variable inflammatory syndrome.  

Biopsy of the skin lesions shows an infiltrate of neutrophilic polymorphonuclear cells

4. Treatment :

Colchicine has an effect on mouth ulcers and joint diseases (first-line treatment for simple cutaneous-mucosal forms). 

It is also effective on skin conditions. 

• Anti-inflammatory steroids (corticosteroids) are the mainstay of treatment for colchicine-resistant or more severe cutaneous forms.
• Immunosuppressants (usually azathioprine, cyclophosphamide, methotrexate, mycophenolate mofetil) may also be used in concomitant administration.

5. Diagnosis of Behçet’s disease by the dentist

• Firstly, the practitioner faced with an oral lesion must know how to recognise the type of primary lesions. Then, in a second step, after identifying the ulceration, he must determine whether the lesion is of the aphthoid type or not.
• In Behçet’s disease, oral ulcers are recurrent. 
• The dentist will still follow a pattern and question the patient about his age, his personal history (such as the presence of any illness, allergies, medical history) and his previous and current treatments. 
• When the patient informs the dentist of the presence of an oral lesion or when the dentist detects one, the practitioner will clarify his questioning by asking the patient about: 

The alteration of his general condition: fever? Weight loss? Painful adenopathy or not? 

– Possible exposure to an infection: herpes simplex virus?

 – Any history of trauma affecting the mouth: either physical (such as biting or brushing teeth or poorly adapted dental appliance) or chemical (the patient taking medication) 

– Oral symptoms and their severity: pain of the lesion? Bleeding? Lesion preceded by a burning sensation? 

– Functional signs such as pain/difficulty speaking or swallowing as well as difficulty eating. 

– The presence of lesions affecting other parts of the body: skin, eye, genital lesions? 

• Local treatments The dentist will mainly prescribe oral gels to be applied to the areas affected by mouth ulcers. (Anesthetic gels, creams and pastes).
•  He will also be able to write a prescription for colchicine. 
• Precautions for Anesthesia: Trauma can be triggers for canker sore outbreaks. 
• Thus, performing an anesthetic injection can cause the appearance of a mouth ulcer. 
•  In the context of a patient with Behçet’s disease, patients can be on AAP and/or AVK. It is not recommended for a patient at risk of hemorrhage to perform loco-regional anesthesia. 
• Amoxicillin alone (Clamoxyl®) or combined with clavulanic acid (Augmentin®): it is not recommended to combine it with methotrexate (risk of overdose of the latter and increase in its hematological toxicity). 
• Azithromycin, clarithromycin and spiramycin: drug interactions of this family of antibiotics are numerous. It is also not recommended to combine macrolides with immunosuppressants. 
• Metronidazole (Flagyl®): precautions must be taken when using it, particularly when combined with AVKs (increased risk of bleeding).
• Doxycycline: caution should be exercised when combining this molecule with AVKs (increased risk of bleeding). 
• Pristinamycin (Pyostacine®): it should not be prescribed in combination with colchicine. 
• Paracetamol: there are precautions for use, particularly with AVKs. 
•  Codeine: Codeine retains its own specific precautions for use. 
•  Tramadol: it is contraindicated in cases of epilepsy not controlled by treatment and it is not recommended to combine it with carbamazepine. 
• The dentist will have to assess the invasive nature or not of the procedure to be performed, take into account the infectious risk cofactors
•  Certain procedures will then require antibiotic prophylaxis, antibiotic therapy or postponement depending on the benefit/risk ratio.
• If Behçet’s disease is suspected, the dentist should refer the patient to an internal medicine or dermatology department. 
• The dentist will ask the patient with Behçet’s disease for the contact details of their treating physician as well as the specialist physicians who are treating them.
• The dentist will have to take into account the impact of this disease on the patient’s quality of life and will have to adapt his therapeutic attitude to the patient’s psychological state. 

  Sensitive teeth react to hot, cold or sweet.
Sensitive teeth react to hot, cold or sweet.
Ceramic crowns perfectly imitate the appearance of natural teeth.
Regular dental care reduces the risk of serious problems.
Impacted teeth can cause pain and require intervention.
Antiseptic mouthwashes help reduce plaque.
Fractured teeth can be repaired with modern techniques.
A balanced diet promotes healthy teeth and gums.
 

Horton’s disease