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Classic Rheumatic Diseases: Assessment and Associated Dental Management Guidelines

RHEUMATOLOGY OVERVIEW, FACTS, AND DISEASE CLASSIFICATION

Rheumatic diseases are chronic conditions that cause significant morbidity and affect the patient’s quality of life. The patient is often on immunosuppressants to control the underlying inflammation and to improve the long-term outcome of the disease.

It is important to remember that immunosuppressants decrease inflammation but increase the incidence of infections. This increased incidence of infections, coupled with the poor oral hygiene and increased dental decay found in this population, stresses the importance of regular dental intervention for these patients.

Arthritis affecting the hands can also make it difficult for the patient to keep up with the routines of daily oral care. Extractions are often needed when the decay is extensive because the patient has neglected going to the dentist. Thus, the patient must incorporate regular dental visits as an important part of the multidisciplinary care to stay dentally healthy.

Classification of Rheumatic Diseases

Rheumatic diseases are classified as follows:

SYSTEMIC LUPUS ERYTHEMATOSUS

Overview

Systemic lupus erythematosus (SLE) is a chronic autoimmune condition associated with a hyperactive immune system that attacks normal tissue. The exact cause for this adverse action is unknown. Systemic lupus is a multisystem disease that can affect the skin, joints, blood, lungs, kidney, heart, brain, and the nervous system. SLE typically affects women in the child-bearing age, and it is postulated that estrogen plays a role with this disease state. Antibody production is important for the diagnosis of SLE.

Eleven Criteria Associated with SLE

To make a diagnosis of SLE at least four of the following eleven criteria must be present:

The LE (lupus erythematosus) cell test is positive in only 50% of patients with SLE. In some cases the patient may be tested for specific ANA subtypes. Anti–double-stranded DNA (anti-ds DNA) is usually found only in SLE patients. Anti-Sm antibodies are also usually found only with SLE. When the ANA is negative and there is a strong suspicion for lupus, the anti-Ro and anti-La antibodies, when detected, can identify a rare form of lupus called Ro lupus.

Symptoms and Signs

No two patients with lupus will have similar symptoms. Symptoms will vary according to the organ systems affected. Additionally, these patients can have alopecia and may present with oral candidiasis due to the underlying xerostomia commonly affecting the majority of these patients. Overwhelming infection and kidney failure are two of the common causes of death in people with lupus.

Treatment

The treatment is aimed at decreasing inflammation with high dose NSAIDS, corticosteroids, and cytotoxic drugs.

Drug Precautions

Patients with lupus are often allergic to multiple drugs. Always go through the allergy history very carefully. Drug history may show that the patient has responded adversely to sulpha antimicrobials and penicillin in the past. All drugs causing even a minimal form of allergy should be avoided.

SCLERODERMA

Scleroderma is an autoimmune condition associated with excessive fibrosis or scar tissue formation involving organs and the skin of the face and distal extremities. Scleroderma causes thickness and firmness of the affected areas. Vascular reactivity causing Raynaud’s phenomenon is quite significant with scleroderma. Auto-antibodies are useful in the diagnosis of the disease.

Forms of Scleroderma

There are two forms of the disease: diffuse scleroderma and limited scleroderma.

Diffuse Scleroderma

Diffuse scleroderma is the more severe form of scleroderma, which begins with thickening of the skin of the trunk and extremities. This rapidly progresses to fibrosis and involvement of vital organs. Heart, lungs, esophagus, and the kidneys can be affected. Renal insufficiency occurs in >10% of patients. Antiscleroderma-70 antibodies are present in 40% of the patients.

Limited Scleroderma/Limited Form (CREST)

This is the more benign form of scleroderma and it affects mainly the skin of the face and the digits. This form progresses slowly. CREST represents the clinical pattern associated with this form and it stands for calcinosis, Raynaud’s phenomenon, esophageal involvement, sclerodactyly, and telangiactasias. There is less cardiac and pulmonary involvement with this form of scleroderma. Anticentromeric antibodies are exhibited by 80% of the patients.

Clinical Features

The scleroderma patient can present with the following features:

Diagnosis

The diagnosis of scleroderma is based on the presence of the clinical features of the disease and specific blood tests. Most scleroderma patients are antinuclear antibody (ANA)–positive.

The CREST or the limited form is exclusively positive for the anticentromere antibody. The Anti-Scl 70 antibody (anti–topo-isomerase I antibody) is positive in most cases of the diffuse form of scleroderma.

Treatment

Treatment is directed toward the organs affected. Special emphasis is placed on the monitoring and control of the blood pressure (BP) to slow the progression of associated kidney disease.

SJÖGREN’S Syndrome

Introduction

Sjögren’s syndrome is a chronic autoimmune condition associated with hypofunction of the lacrimal glands, causing dry eyes or keratoconjunctivitis sicca and hypofunction of the salivary glands, which causes xerostomia.

The “old” Sjögren’s syndrome (SS) classification that is now considered obsolete was based on the presence or absence of other autoimmune conditions with SS. It was classified as “Primary,” when the autoimmune condition was present alone and “Secondary” when the condition was present with other autoimmune conditions such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), or scleroderma.

The “new” American-European classification system for the diagnosis of primary Sjögren’s syndrome is based on the fulfillment of objective criteria. It requires the patient to have four of the six criteria, and either criterion number 5 or criterion number 6 must be met. In the Sjögren’s syndrome patient that has no sicca symptoms, the diagnosis of SS can be made if the patient has met three of the four objective criteria (criteria numbers 3–6).

Criteria

The six diagnostic criteria are as follows:

Sjögren’s syndrome patients need coordinated care provided by experts in the fields of oral health, rheumatology, and ophthalmology to achieve the optimal evaluation and management of this condition. Treatment is largely based on symptoms, and the patient should be monitored for the potential development of lymphoma.

Most Sjögren’s syndrome patients present with sicca symptoms: xerophthalmia (dry eyes), xerostomia (dry mouth), and parotid gland enlargement. Additionally, the patient may also have arthralgia, arthritis, Raynaud’s phenomenon, myalgia, pulmonary disease, gastrointestinal disease, leucopenia, anemia, lymphadenopathy, neuropathy, vasculitis, renal tubular acidosis, and lymphoma. Almost 50% of patients with SS have cutaneous findings such as dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria.

Complications

General complications associated with SS are:

• Other autoimmune conditions: There can be an emergence of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA).
• Lacrimal gland occlusion: Occlusion of the lacrimal puncta is corrected surgically by electrocautery and other techniques for permanent punctal occlusion.
• Parotid gland infection: Typically staphylococcal, streptococcal, or pneumococcal infection can occur. The patient can present with unilateral worsening of symptoms, along with tenderness, warmth, and erythema. An elevated amylase level may occur from parotid gland involvement.
• Parotid tumors: The provider should always be on the lookout for any new unusually hard or unilateral parotid enlargement.
• Pregnancy outcome: Pregnant patients with a positive SS-related blood test are at risk for fetal loss, complete heart block in the fetus, and neonatal lupus syndrome in the newborn.
• Lymphomas: The patient can present with pseudo-lymphomas, showing pleomorphic cells, and non-Hodgkin B-cell lymphomas.

Xerostomia-Associated Oral Side Effects and Dental Alerts

Side effects and dental alerts include:

• Inability to eat crackers because they stick to the roof the mouth.
• The tongue often sticks to the roof of the mouth.
• Drinking water at night, resulting in nocturia.
• Difficulty speaking for long periods of time or of hoarseness.
• Higher incidence of dental caries and periodontal disease, which warrants more frequent visits to the dentist and hygienist.
• Altered sense of taste.
• Difficulty wearing dentures due to xerostomia, and the patient needing frequent dentire adjustments.
• Development of oral candidiasis with painful angular cheilitis.
• Lack of saliva may lead to impaired clearance of acid, and this may result in gastroesophageal reflux and esophagitis.
• Patients with Sjögren’s syndrome are at increased risk for delayed gastric emptying, upper abdominal discomfort, nausea, and vomiting. Always have the patient seated in a semi-sitting position in the chair.
• Cranial neuropathies, particularly trigeminal neuropathy, or facial nerve palsy can develop along with CNS involvement in general. Avoid using the 4% local anesthetics in SS patients.
• A progressively elevated erythrocyte sedimentation rate (ESR) indicates active/ progressive inflammation, and this warrants consultation with the patient’s physician. Routine dentistry must be temporarily deferred.
• CBC may show anemia, leucopenia, and/or eosinophilia. Pernicious anemia may be associated with the atrophic gastritis. An abnormal WBC, count, especially with an abnormal differential count, should trigger concerns for a lympho-reticular malignancy. In addition, although a low platelet or WBC count can occur in persons with primary Sjögren’s syndrome, the finding should also prompt the provider to consider coexisting SLE. Half of the SS patients present with mild, normochromic, normocytic anemia, and leukopenia occurs in almost half of the SS patients. Implement appropriate anemia or ANC-associated guidelines, when required.
• Renal stones, progressive weakness, and paralysis secondary to hypokalemia from underlying renal tubular acidosis and osteomalacia are not uncommon with SS. Severe hypokalemia can lead to periodic paralysis. Renal tubular acidosis can also be associated with a low bicarbonate level. Always obtain serum creatinine and electrolytes prior to dentistry, and implement appropriate deviations in AAAs in the presence of decreased renal function.
• High total protein level or a low albumin level should prompt the clinician to perform serum protein electrophoresis
• The patient can have high alkaline phosphatase level due to associated primary biliary cirrhosis that commonly affects SS patients.
• Chronic active hepatitis or hepatitis C can also occur with SS, causing an elevated transaminase levels. Mild (less than twofold) increases in transaminase levels are not uncommon with SS, so always obtain LFTs prior to dentistry.

Prognosis

Per say, the prognosis for Sjögren’s syndrome is good if the patient does not develop a lympho-proliferative disorder. Declining exocrine gland function from infiltration with lymphocytes does increase the morbidity associated with Sjögren’s syndrome. The SS-associated mortality rate increases when disorders such as RA, SLE, primary biliary cirrhosis, and/or lymphoma occur, which cause a decline in function.

Treatment

No curative agents for Sjögren’s syndrome exist. The treatment of the disorder is essentially symptomatic. In secondary Sjögren’s syndrome, treatment is based on the accompanying disease and its clinical features. Sjögren’s syndrome and associated SLE improve more than primary Sjögren’s syndrome. In Sjögren’s syndrome associated with polymyositis, monthly cyclophosphamide pulse therapy has been successful. Long-term anticoagulation may be needed in patients with vascular thrombosis related to antiphospholipid antibody syndrome. Among the biologic therapies, etanercept has failed to demonstrate benefit in Sjögren’s syndrome, whereas rituximab appears promising in the treatment of vasculitis and intravenous immunoglobulin (IVIG)–dependent ataxic neuropathy.

Dry Mouth Therapy

Dry mouth therapy consists of:

• Frequent sips of water.
• Sugar-free lemon drops to stimulate salivary secretion.
• Patients should avoid medications with anticholinergic and antihistamine effects.
• Humidifier use can possibly help.
• Regular dental check ups and fluoride treatments.
• Use toothpaste without mouth irritation: Recommend Biotene toothpaste, Biotene mouth rinse, Dental Care toothpaste, and Oral Balance gel.
• Assess for oral candidiasis and angular cheilitis. Treat infection with topical antifungal such as nystatin troches, but oral fluconazole may occasionally be needed. Denture wearers should disinfect their dentures.
• Treat all sinus-related issues immediately because these issues, when untreated, can promote mouth breathing and worsening of xerostomia.
• The patient should use isotonic sodium chloride solution nasal sprays and avoid antihistamine use.

Topical and Systemic Therapies

Topical and systemic therapies are also discussed in Chapter 48. The reader is advised to review those contents and the contents discussed here.

Topical Therapies

Artificial saliva preparations contain methylcellulose, sorbitol, and salts to moisten and lubricate the mouth. Topical therapy for Sjögren’s syndrome should be prioritized, but pilocarpine or cevimeline should be opted for treatment of xerostomia when local therapy is not successful.

Common OTC artificial saliva preparations:

Systemic Therapies

Other Therapies

REITER’S SYNDROME

Reiter’s syndrome or reactive arthritis is an autoimmune condition that affects white males, most commonly, ages 20–40. It occurs in response to a genitourinary or gastrointestinal infection. It is an HLA-B27–linked inflammatory arthritis that damages the cartilage of the joints, which triggers subacute or chronic inflammatory disease. The inflammatory response triggers a triad of symptoms: arthritis, uveitis, and urethritis.

Characteristic Features

The characteristic features associated with Reiter’s syndrome are conjunctivitis, nonspecific urethritis, arthritis affecting mostly the joints of the lower extremities (feet and heels), and painless, shallow oral and genital ulcers.

Treatment

Treatment consists of providing adequate antibiotics to treat the precipitating infection, NSAIDS, and corticosteroids.

BEHÇET’S SYNDROME

Behçet’s syndrome, or Behçet’s disease, is associated with inflammation of the blood vessels. Behçet’s syndrome affects patients who are 20–30 years old. The disease is chronic and recurrent with asymptomatic periods in between, but the flare-ups are unpredictable. It is prevalent in North Africa, Turkey, the Middle East, Korea, and Japan.

Clinical Features

Behçet’s syndrome is characterized as follows:

Behçet’s Syndrome Treatment

Treatment is symptomatic and focuses on pain relief and prevention of serious problems.

TEMPORAL OR GIANT CELL ARTERITIS

Temporal or giant cell arteritis is a vasculitis that affects medium and large blood vessels, particularly in the head. It is called temporal arteritis because the temporal artery is frequently affected. The alternate name, giant cell arteritis reflects the cells seen on biopsy of the affected blood vessel.

It is not a permanent disease because it completely disappears in 1–2 years. The disease affects the elderly, and women are more affected than men. It is rare before age 50 and is most common around age 70.

Classic Features

The patient typically complains of temporal headache that is associated with a tender, thickened, and nodular temporal artery. However, temporal arteritis can also affect other vessels such as the opthalmic, facial, or lingual arteries. Ophthalmic and posterior ciliary arteries, when involved, can lead to blindness.

Facial artery involvement is rare, but when it occurs it is associated with painful jaw swelling or claudication. Lingual artery involvement is rare and when affected it causes color change, paresthesias, and necrosis of the tongue. Oral pain may be prominent at the level of the gums.

Diagnostic Test

Temporal or giant cell arteritis characteristically shows immediate elevation of the ESR with values rising at the rate of 100mm/h. Definitive diagnosis is established only with tissue biopsy of the affected vessel.

Treatment

Treatment consists of high-dose corticosteroids, which rapidly correct the symptoms and prevent blindness. Steroids should be given immediately even before the return of biopsy results if the suspicion is high. The corticosteroids are continued if the biopsy results are positive and discontinued with negative results. Once started, the steroids are gradually decreased over months.

RHEUMATOID ARTHRITIS

Rheumatoid arthritis (RA) affects more women than men and occurs around age 30 or older.

Clinical Features

The patient experiences symmetrical inflammation of the distal joints: digits, wrists, feet, and knees. The pain in the joints is worst upon waking up. The knuckles and the proximal interphalangeal joints of the hands and feet are affected. The cervical spine is involved in severe cases, but involvement of the spine is rare. Lumbar spine is always spared. The patient complains of prolonged morning stiffness, malaise, and sometimes a low-grade fever.

Cervical Spine Involvement

RA-associated cervical spine changes are prominent at the uncovertebral joints and bursae. Uncovertebral joints are small synovial joints formed secondarily between the lateral lips (uncinate processes) of the superior surfaces of the bodies of the lower cervical vertebrae, and the inferior surface of the superior vertebral body. The synovial bursae surrounding the dens are often affected, causing lysis and disruption of the transverse ligament of atlas and atlantoaxial subluxation. This can lead to impingement of the spinal cord. Cervical spine involvement is associated with the Lehrmitte’s sign in which the patient experiences flashes of pain in all four extremities.

Cervical Spine Involvement Diagnosis

RA-associated cervical spine involvement is diagnosed with review of the cervical spine films taken with the neck in forward flexion. Diagnosis is confirmed by identifying an increased distance between the dens and the anterior arch of atlas.

Cervical Spine Involvement Treatment

Treatment consists of prompt surgical stabilization and consists of wiring of the C-1 and C-2 posterior arches.

Cricoaritenoid Arthritis

This is a frequent manifestation of RA. The patient experiences pain, dysphagia, fullness or tension in the throat, hoarseness, and stridor.

Rheumatoid Arthritis Blood Tests

CBC often shows thrombocytopenia and anemia in patients with RA.

Rheumatoid Arthritis Treatment

Treatment is symptomatic and focuses on pain relief: NSAIDS, corticosteroids, DMARDs, and chemotherapeutic drugs. Please also refer to the “Antirheumatic Medications” section in this chapter for detailed discussion of RA drug therapy.

Tocilizumab (Actemra) is a newly approved drug used to treat adult patients with moderately-to-severely active RA, who have had an inadequate response to tumor necrosis factor (TNF)-alfa inhibitors, as well as patients who have not responded well to methotrexate and other oral DMARDS.

Tocilizumab (Actemra) adverse effects: Tocilizumab use could be associated with upper airway infection, hypertension, hepatotoxicity as evidenced by an increased ALT, neutropenia, thrombocytopenia, demyelinating disorders, lipid abnormalities potentially precipitating cardiac disease, neurotoxicity, and gastrointestinal perforation. The provider must always assess the CBC with ANC counts prior to dentistry if the patient is on tocilizumab. Tuberculosis (TB), invasive bacterial, viral, and fungal opportunistic infections can occur with tocilizumab (Actemra). The drug is interrupted temporarily if a serious infection develops.

OSTEOARTHRITIS

Facts

Osteoarthritis is differentiated from RA in that osteoarthritis/>