Gougerot-Sjögren syndrome

Gougerot Sjogren

1. Gougerot-Sjögren syndrome: 

•  Called Sjögren’s syndrome by the Anglo-Saxons, it is an autoimmune disease, that is to say a disease which is linked to an excess of immunity. 
• This “hyperimmunity” during Sjögren’s syndrome can lead to inflammation of different tissues or organs, such as the salivary and lacrimal glands (which produce saliva and tears), other organs such as the lungs, kidneys and nerves. 
• Sjögren’s syndrome can exist in isolation (primary Sjögren’s syndrome) or associated with other autoimmune diseases (such as lupus, rheumatoid arthritis, scleroderma or others), in which case we speak of associated or secondary Sjögren’s syndrome. 
• Sjögren’s syndrome is named after a French physician, Henri Gougerot, and a Swedish ophthalmologist, Henrik Sjögren, who were the first to describe the disease.

2. EPIDEMIOLOGY

These are most often women between 40 and 60 years old (the sex ratio is in favor of women with 9 women for 1 man affected). 

 The disease can appear earlier, sometimes even in children.

3. CLINICAL MANIFESTATIONS

Most often, the disease begins with a 

• dry eyes and mouth, 
•  joint pain and severe fatigue. 
•  This great fatigue is not linked to serious damage to an organ but to a fragile general condition. 
•  Dry mouth and eyes may include a feeling of having sand or cotton in the mouth or a “pasty” mouth, sand in the eyes, and sticky eyes (as with conjunctivitis). 
•  Dryness of the nose and airways can result in a dry cough. 
•   The skin and vagina may also be dry.
• Joint damage results in joint pain and sometimes swelling.
• Nerve damage manifests itself as sensations of electric shock or tingling, rarely with a lack of strength.
•  Lung involvement may show no signs or may result in emphysema (dilation of the pulmonary alveoli) 
•  Involvement of the kidney, brain, muscle or other organs is extremely rare.
•  In 5% of patients, lymphoma (a malignant tumor of lymphatic cells) may occur, most often in the main salivary gland (the parotid). This is most often a cancer with a good prognosis if detected early.

4. DIAGNOSIS

To be sure of having Gougerot-Sjögren syndrome you must: 

– Assess dry eye using a strip (Schirmer test) or dyes (lissamine green or fluorescein test)

– Assess dry mouth by measuring saliva flow (repeated spitting into a tube for 5 minutes)

• Doing blood tests to look for certain antibodies like anti-SSA/anti-SSB antibodies is helpful.
•  Some patients also have a high level of antibodies (polyclonal hypergammaglobulinemia) and, more rarely, a small drop in white blood cells (especially lymphocytes).
•  A biopsy of the accessory salivary glands is a minor surgical procedure, performed under local anesthesia, which allows the small glands of the lip to be examined under a microscope and signs of inflammation to be looked for. 

5. Differential diagnosis 

There are many causes of dry eye syndrome, the main ones being:

 • Medications: imipramine antidepressants, neuroleptics, antiparkinsonians, atropine, morphine, antihistamines, antiacne drugs, etc.

 • Physiological aging

 • Postmenopausal estrogen deficiency 

• Prolonged wear of contact lenses 

• Unbalanced diabetes 

• Cervicofacial radiotherapy

 • Amyloidosis 

• Sarcoidosis 

• Graft versus host disease 

• Certain viral infections (HIV, HCV). 

6. TREATMENTS

Treating dry mouth: pitfalls and solutions

More than 20% of our patients complain of xerostomia, which is the sensation of having a dry mouth.

 Medications, particularly anxiolytics, age, tobacco, autoimmune diseases with Gougerot Sjögren’s disease in the lead or even cervico-facial irradiation, are associated with a decrease in salivary production and constitute the main risk factors.

• Saliva is a major protective factor for our teeth. 
• It is made up of 99% water but also proteins, mineral salts, desquamated cells, and defense factors (immunoglobulins, antimicrobial peptides). 
• Distributed in a thin film on the tissues of the mouth (teeth, periodontium, mucosa), it is an essential element of its balance: control of pathogenic flora, healing, tissue lubrication, buffering power, remineralization of teeth. 
• It is therefore easy to understand that the reduction in salivary flow is associated with an increased risk of dental diseases (cavities, tooth wear), gingival inflammation, or even oral candidiasis, but also oral discomfort and eating difficulties. 

7. Support

• It is first of all  medical care  which allows us to regain a certain control over the imbalance.

       – Recognizing and simply explaining the role of saliva and the consequences of its reduction is a first step which reassures the patient.

– Advising actions and attitudes to improve daily life helps make this constant discomfort more acceptable.

– Prescribing saliva stimulants is possible provided you know them well.

– Prescribing fluoride in suitable forms (toothpaste, mouthguard, varnish) is essential to compensate for weak natural defenses.

• Dental and periodontal treatments are   very often necessary. They must take into account the specific nature of the often multiple, severe and recurring damage. Comprehensive and rapidly implemented care, in the form of sectoral treatment, is an interesting solution. Tissue preservation also requires different thinking.
• Follow-up visits twice a year are necessary.
• It seems important to understand the particularity of these patients. The success of the treatment can only come through work on daily balance, comprehensive and lasting support and an awareness of the complexity of treatments.

Treatment : 

• Teaching the patient about environmental factors to avoid: smoke, wind, dry surroundings, prolonged reading 
•  Avoidance of medications causing dry syndrome 
•  Treatment of xerophthalmia: artificial tears, viscous polymers, carbomer or hyaluronic acid gels and topical cyclosporine 
•  Treatment of xerostomia: local saliva substitutes or intraoral devices and sialagogues (Pilocarpine 5mg/4 times a day)
•  Oral hygiene methods adapted to the increased risk of tooth decay: use of unsweetened beverages and sugar-free chewing gum, oral care with a low-pressure water jet, dental floss and twice-daily brushing of teeth 
•  Anti-fungal treatment for oral candidiasis. 

Gougerot-Sjögren syndrome

PERIODONTAL DISEASES

• The periodontium (from the Greek para = next to; from the Latin odus = tooth) is an organ whose role is to keep the teeth firmly and durably attached to the jaws and to connect them to the rest of the body.
• It is made up of four connective tissues: 
• Periodontal diseases are infectious diseases, of a mixed type, with a predominance of anaerobic bacteria. 
• They are multifactorial, due to an imbalance in the oral ecosystem. Classically, they are divided into gingivitis and periodontitis. 
• Gingivitis: infectious diseases with a non-specific, reversible inflammatory reaction of the marginal gingiva, in response to an increase in the number of bacteria present in the sulci. 
•  Periodontitis: This is the result of an inflammatory response (acute or chronic) of the deeper tissues of the periodontium to the excessive presence or virulence of certain species (essentially Gram-negative) in the gingival sulcus. 
• Clinically, the more or less significant destruction of these tissues leads to the more or less rapid loss of the epithelial-connective attachment, and sometimes results in the avulsion of the teeth. 
• Depending on the speed of progression of the disease and the quality of the periodontal tissues, a periodontal pocket may form.
•  The disease alternates between latency phases and acute phases of inflammation. 
• Gougerot-Sjögren syndrome, due to the significant reduction in salivary flow that it causes, increases the risk of damage to oral tissues. 
•  The starting point is the drying up of secretions from the salivary glands. 
• This has the effect of altering the buffering capacity of saliva, which is so important in protecting against demineralization of dental tissues. 
• The reduced salivary pH then modifies the oral flora with the appearance of cariogenic bacteria. The caries risk is therefore greater in these patients. 
• Premature tooth loss is also a feature of SS and this begins even before the onset of symptoms. At the periodontal level, although results diverge in the literature, inflammation is more marked in pSS patients.
•  There is still no curative treatment for this disease, which is why it is essential to convince patients with SS of the importance of prevention in order to limit the oral-dental consequences of the disease as much as possible. 

Treatment of dry mouth

• Important for comfort and dental health 
• Good hydration
• Drink water or sugar-free liquids regularly 
• Avoid sugars, carbonated drinks, juices and “waters” with additives 
• Sugar-free saliva stimulants
• Avoid medications that can make dryness worse
• Humidifiers

• Important for comfort and dental health
• Preventative dental care and be managed by a dental hygienist and dentist with experience in dry mouth care whenever possible. 
• Flossing after meals 
• Fluoride (toothpaste or mouthwash) 
• Use toothpastes specially designed for dry mouth. 

• Artificial saliva (glandosan) for temporary relief of symptoms 
• Systemic stimulants

               Pilocarpine (salagen) 5 mg orally, four times/day 

               Cevimeline 

               Sulfarlem 

• Recognition and treatment of oral candidiasis (pain, burning sensations, hypersensitivity, white lesions of the mucous membranes).
• Systemic immunosuppressants? 

Gougerot-Sjögren syndrome

Polyarteritis nodosa

I- Epidemiology:

• Rarer than Horton’s disease: 3-4/10 ⁶ /year
• Affects all ages, more common between 40 and 60 years old.

II- Anatomy pathology:

• Fibrinoid necrosis of the media: anhist, acellular substance, protein debris
• Major lymphoplasmacytic infiltrate
• Eosinophils
• Absence of giant cells.

III- Clinical manifestations: 

• General syndrome: often very marked 

Massive weight loss

Fever, poorly tolerated, various appearances

Severe myalgia, pain on muscle pressure

Arthralgia

True inflammatory arthritis

• Neurological signs:

Peripheral involvement: multiple neuritis, rather distal, with rapid onset in the upper and lower limbs 

Secondary amyotrophy

Successive thrusts 

Very slow recovery if treated, inevitable worsening if not treated.    

• Muscle manifestations: 

Affects muscular arterioles: explains intense myalgia, can lead to muscular atrophy

Accessible to biopsy, quadriceps; possibly directed by EMG (electromyogram). 

Muscle enzymes usually normal 

• Joint manifestations:

Arthralgia

True arthritis, often asymmetrical

Rather the large joints of the lower limbs

• Skin conditions:

Petechial vascular purpura

Hemorrhagic blisters

Livedo frequent

Inflammatory nodules

Distal gangrene

• Kidney damage:

Often fearsome, with very rapid deterioration of renal function

Renal arteriole: renal infarction, hypertension, systemic complications of hypertension

Therapeutic emergency.

• Digestive symptoms: 

Ischemic colitis

Digestive hemorrhage

Digestive perforations

False appendicitis

False cholecystitis

Pancreatitis.

• Heart attack:

Coronary artery disease

Heart failure secondary to malignant renovascular hypertension

Corticosteroid-sensitive dilated cardiomyopathy.

Pericarditis: not serious.

IV- ACR diagnostic criteria: 

Weight loss > 4 kg 

Livedo

Testicular pain

Myalgia, fatigue or leg pain

Mono or polyneuropathies

Diastolic BP > 90 mm Hg

Creatinine > 130 µmols/l

Hepatitis B virus

Aneurysm or occlusion of visceral arteries on arteriography

Positive biopsy of small or medium caliber arteries 

3/10 criteria necessary for diagnosis  

V- biology:

• Inflammatory syndrome: VS, CRP
• ANCA: in less than 20% of cases
• Systematic search for HBV Ag and Ac.
• However, in less than 10% of cases, there is an association with a virus such as hepatitis B (HBV), hepatitis C virus (HCV), parvovirus or even human immunodeficiency virus (HIV). 

Gougerot-Sjögren syndrome

VI- Oral facial manifestations

• In the mouth, we sometimes note gingivitis , ulcerations, isolated or multiple nodules or even small hemorrhages. 

7. Treatment

Treatment of inflammatory syndrome Corticosteroids (or corticosteroids), including prednisone and prednisolone, are anti-inflammatory drugs that can control the disease quickly.

Association with an immunosuppressant is necessary. 

Cyclophosphamide is the most commonly used immunosuppressant to treat PAN.

• High blood pressure, which is common in people with PAN, can be controlled with medications such as enalapril. 
• Sometimes treatment with methotrexate. 
• Cutaneous periarteritis nodosa in adults regressive after treatment of dental infections. 
• Therefore, the doctor prescribing anti-TNFα, before initiating treatment, can request a dental check-up with dental panoramic from the dental surgeon. 
•  The practitioner must explain to the patient the need for examinations and care aimed at eliminating any potential source of infection and emphasize the importance of optimal oral hygiene. 
•  As routine dental care (cavities, scaling) carries a lower risk of infection, they do not require stopping anti-TNFα treatment. However, preventive antibiotics may be prescribed depending on the case (background, age, illness). 
• The dentist should contact the specialist doctor in charge of anti-TNFα treatment who, if deemed necessary, will decide to temporarily stop the treatment. The implementation of antibiotic prophylaxis or not will also be discussed. 
• The dentist must remind his patient of some practical rules to avoid certain sources of infection: 

•  Having good dental hygiene is important to avoid any oral infections, as is changing your toothbrush regularly and, if necessary, disinfecting it.
•  Hand washing and personal hygiene are essential against infections. 
• The combination of methotrexate and nonsteroidal anti-inflammatory drugs causes an increase in the hematological toxicity of methotrexate (decrease in renal clearance of methotrexate by anti-inflammatory drugs). Its combination is contraindicated with phenylbutazone (BUTAZOLIDINE®). 

  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.
 

Gougerot-Sjögren syndrome