Dental Considerations for Patients with Lupus Erythematosus

Dr. Phyo Ko Ko
Roll No. 9 (Group V) Dip.D.Sc. candidate (12/2012)

Lupus Erythematosus

Immunologically mediated condition so called collagen vascular or connective tissue diseases Several clinicopathologic forms
Systemic lupus erythematosus ( SLE ) Discoid lupus erythematosus ( DLE ) or Chronic cutaneous lupus erythematosus ( CCLE )
3.

1. 2.

Subacute cutaneous lupus erythematosus

Systemic Lupus Erythematosus ( SLE )

Is the prototypic multisystem autoimmune disorder with a broad

spectrum of clinical presentations encompassing almost all organs and tissues

Hallmark feature chronic inflammation Can affect

Skin Joints Kidneys Lungs Nervous system Serous membrane such as pleura and pericardium Mucous membrane Other organs of the body

 Women ( age between 30 40 years ) are affected more

frequently than Man

Average ratio ~

10 : 1 between 12 and 50 per 100,000

Worldwide prevalence Clinical course

episodes of recurrent acute or chronic inflammation, and intervening periods of remission

Lupus flare
Flare can be considered as a reappearance of clinical features which

were earlier quiescent

Certainly easily identifiable and avoidable triggers for lupus flare

1. Exposure to sunlight 2. Physical and mental stress 3. Intercurrent infections 4. Pregnancy 5. Non compliance with treatment or sudden withdrawal of drugs

Clinical features which can be considered as a warning of impending flare:

Increasing fatigue Arthralgias and myalgias New or worsening rash Persistent headache Fever Abdominal pain

Aetiology
Genetic factors and specific gene loci Environmental factors

- exposure to sunlight ( photosensitivity ) - drugs ( pharmacogenetics ) - infections ( Epstein-Barr virus )

These factors lead to an irreversible break in immunological

tolerance manifested by immune responses against endogenous nuclear antigens

Pathogenesis
Type III Hypersensitivity reaction

may be generalized or organ specific

Appropriate antigen (+)formation of soluble immune complexes

(endogeneous nuclear antigen/autoantigen)

(mainly composed of IgG and IgM)

Inflammatory response & tissue damage

Type III Hypersensitivity

Aberrant apoptosis or insufficient clearance of apoptotic cells

Apoptotic blebs and nucleosomes

These taken up by immature myeloid dendtritic cells (mDCs)

Mature mDCs produces proinflammatory cytokines such as IL-6

Activation of Helper T cells (Th-1, Th-2) and Inhibition of Regulatory T cells (Tregs)

Activated B cells

Autoreactive B cells produce autoantibodies

Autoantibodies + Apoptotic material

Immune complexes

These were taken up by Plasmacytoid dendritic cells (pDCs) and produce

INF- which enchances autoantibodies production and isotype switching

Result in increasing concentrations of immune complexes

EAC early apoptotic cell M macrophage ACB apoptotic cell body mDC myeloid dendritic cell Th helper T cell IL interleukin Treg regulatory T cell pDC plasmacytoid dendritic cell IFN tumour necrosis factor

Clinical Presentation
Constitutional symptoms

complaint of - fatigue - malaise - arthralgia - myalgia - mucocutaneous lesisons

1. Musculoskeletal features
Artharalgia

- earliest manifestation - asymmetric and migratory - joints of hands are mostly affected
Arthritis

- small joints of hands, wrists and knees

Tendon involvement deformities Myositis

- generalized myalgia and muscle tenderness involving proximal muscles

2. Mucocutaneous features Skin lesions

Classic malar or butterfly rash

-presents acutely as an erythematous, elevated lesion, pruritic or painful in malar distribution -may last from days to weeks -that spares the nasolabial crease
Photosensitivity development of rash after exposure to UVB,

UVA radiation from sunlight or fluorescent lights

Malar or Butterfly rash

Oral lesions
25 45 % of SLE patients Irregularly shaped raised white plaques areas of erythema silvery white scarred lesions, and Ulcers with surrounding erythema Soft or hard palate and buccal mucosa Usually painless Advance cases

may have features of Sjgrens Syndrome

Histopathology of Oral lesions

Microscopic features are quite similar to those of lichen planus

and erythema multiforme

Common feature

band like subepithelial inflammation

In SLE and EM, inflammatory infiltrate extends deeper into the Lupus lesion will exhibit periodic acid-Schiff staining in

underlying connective tissue and shows a perivascular pattern basement membrane zone

Direct immunofluorescent testing

immunoglobulin and complement deposition along basement membrane zone

3. Renal features
30 % of patients Immune complex deposition intra-glomerular inflammation Lupus nephritis

Proteinuria

( grave complications of SLE )

To detect and monitor disease renal activity Urinalysis Chronic renal failure

influence the choice or dosage of

medications

4. Nervous System features

Headache Depression Seizures SLE affects both CNS and PNS Dentists role to rule out odontogenic, TMJ and associated

Psychosis Peripheral neuropathies Migraines

myofacial sources of pain

5. Pleura and Lungs

Pleuritis

most common manifestation

Pleuritic pain ( generalized chest pain aggravated by deep

inspiration)

Cough and rapid shallow breathing Plueral effusion Parenchymal damage

lead to pneumonitis great risk of acquiring

Hospitalized patient with SLE

pneumonia

6. Cardiovascular features
Pericarditis Pericardial effusion may be asymptomatic Myocardial involvement is rare Patient may present with fever, dyspnea, tachycardia and

congestive heart failure

Valvular heart disease ( most common abnormality is diffuse

thickening of mitral and aortic valves followed by vegetation, valvular regurgitation and stenosis )

 Endocarditis

autoimmune basis

resulting rheumatic valvular damage

in a form of sterile vegetation

Libman-Sacks endocarditis/vegetation Antibiotic prophylaxis is required to prevent infectious

endocarditis in SLE patients with valvular damage

 Advanced SLE-induced vasculitis can lead to multiorgan

dysfunction

Raynauds phenomenon

color

cold- or stress-induced triphasic

changes of the hands and feet

Only 8 % of SLE patients have inflammation of retinal artery

Nailfold vasculitis

Raynauds phenomenon

7. Hematological features
Major clinical manifestations Anaemia

due to autoimmune haemolysis

Leucopenia

WBC count

< 4500/mm3

Lymphocytopenia ( lymphocyte count < 1500/mm3 )

thrombocytopenia

usually mild ( platelet count 100000 150000 / mm3 )

8. Lymphadenopathy and Splenomegaly

lymphadenopathy

- 40% of patients - usually at onset of disease and during disease flares - soft, non-tender, discrete in cervical, axillary and inguinal nodes - rarely > 2cm
Splenomegaly

- 10 45 % of patients

9. Liver and GI tract features

GI manifestations

- presents in 25-40% of patients - Dyspepsia - Peptic ulcer - Abdominal pain accompanied by nausea and vomitting
Hepatomegaly

- 12 25 % of patients

Frequency of various manifestations of SLE

Manifestations Arthralgia Constitutional Skin Arthritis Renal Raynauds CNS Vasculitis Mucous membrane Gastrointestinal Onset (%) 77 53 53 44 38 33 24 23 21 18 Anytime (%) 85 77 78 63 74 60 54 56 52 45

Manifestations Lymphadenopathy Pleurisy Pericarditis Lung Nephrotic syndrome Azotaemia Myositis Thrombophlebitis Myocarditis Pancreatitis

Onset (%) 16 16 13 7 5 3 3 2 1 1

Anytime (%) 32 30 23 14 11 8 3 6 3 2

Chronic cutaneous ( discoid ) lupus erythematosus

Mild form of lupus Skin lesion

discrete, erythematous, slightly infiltrated plaque covered by wellformed adherent scale that extends into dilated hair follicles (follicular plugging)
Often seen on the face, neck, and scalp leave depressed central scars, atrophy, telangiectasias, and

dyspigmentation

renal, cardiac, and cerebral disease are usually not present

Discoid lupus erythematosus

Discoid oral lesion

Subacute cutaneous lupus erythematosus

Intermediate form of lupus Skin lesions are mild without discoid appearance, do not scar

common affected areas shoulders, forearms, neck and upper torso

With some degree of musculoskeletal involvement renal, cardiac, and cerebral disease are usually not present

Subacute cutaneous lupus erythematosus

Diagnosis
The diagnosis of lupus requires integration of patients

symptoms, physical examination findings, and the results of diagnostic tests or investigations. criteria for diagnosis of lupus more of these criteria

The American College of Rheumatology has designated 11 To receive the diagnosis of lupus, a person must have 4 or

American College of Rheumatology Diagnostic Criteria for SLE , 1997 (revised Tan and others 1982)
SLE criterion Malar ( butterfly ) rash Discoid rash Photosensitivity Oral ulcers Arthritis Serositis Renal disorder Definition or Examples Fixed erythema over the malar eminences Erythematosus raised patches, may scar Skin rash as a result of unusual reaction to sunlight Often painless sores Nonerosive: Jaccouds arthropathy Pleuritis pleuritic pain, pleural rub, pleural effusion Pericarditis ECG changes, pericardial rub, pericardial effusion Proteinuria ( with 3+ or more protein noted in urinalysis specimen or 0.5 g of protein/day ) Cellular casts in urine Seizures Psychosis Hemolytic anemia Leukopenia Lymphopenia Thrombocytopenia Anti-DNA antibodies Anti-Sm antibodies Antiphospholipid antibodies Antibodies to nuclear constituents

Neurological disorder Hematological disorder

Immunological disorder

Anti-nuclear antibibody

If diagnosis of SLE is suspected,

Serologic tests Complete blood count with differential white blood cell counts Disease-specific tests for autoantibodies

- antinuclear antibody - antibody to double-stranded DNA - anti-Smith antibody - Anti-Ro antibody - Antiphospholipid antibody

Autoantibodies found in patients with SLE and their significance

Antibody Antinuclear antibody Antibody to double-stranded DNS Anti-Smith antibody Anti-Ro antibody Antiphospholipid antibody

Significance Indicative of rheumatic diseases Not specific for SLE Suggestive of SLE Predictive for renal involvement Predictive for renal involvement Suggestive for secondary Sjgrens syndrome Increased risk of thromboembolism

Treatment
Goals of SLE management are based on
Prevention Reversal of inflammation Maintaining states of remission and Alleviation of symptoms Avoidance of flare-up of lupus and skin lesions

Medication used in SLE

Nonsteroidal anti-inflammatory drugs (NSAIDs) Cyclooxygenase-2 ( Cox-2 ) selective inhibitors Anti-malarials ( such as hydroxycholoroquine ) Systemic corticosteroids ( such as prednisone )

for patients with mobid symptoms associated with significant organ involvement
Cytotoxic drugs

Dental considerations
Oral lesions Long-term corticosteroids therapy & Adrenal suppression Infection Hematologic abnormalities Cardiac diseases Renal diseases Drugs

Considerations for oral lesions

Oral manifestations of LE may be difficult from lichen planus Immunofluorescence test

PAS-positive thickening of vascular membranes and broad PAS-positive subepithelial band (lupus band)
The drug of first choice is the antimalarial

( Hydroxychloroquine )

Topical steroid creams

Adrenal suppression
Patients with SLE may be taking adrenal suppressive dose of

corticosteroids susceptible to shock months

Glucocorticoid therapy will cause adrenal suppression for up to 12 If previous systemic steroid usage was ceased > 14-30 days, no need

for replacement therapy dose

Lasted >2 weeks and ceased <14-30 days, give previous maintenance Current systemic steroid use, the dose will need to be doubled on the

day of treatment

Patients primary care physician should be consulted

Infection
Patient who are taking cytotoxic or immunosuppressive agents

are at an increased risk of infection.

Impaired immune functions in SLE may predispose to infection Patients with absolute neutrophil count of between 500-1000

cells/mm3 will need perioperative prophylactic antibiotics

Haematological abnormalities
Patient with SLE can frequently develop normochromic

normocytic anemia, hemolytic anemia, leukopenia, and thrombocytopenia. may lead to abnormal bleeding.

Periodontal surgery or extraction of teeth in these conditions Therefore, preoperative complete blood count, prothrombin

time and partial thromboplastin time measurement should be performed prior to any extensive dental procedures.

Cardiac involvement
Libman-Sacks vegetations may occur in SLE patients These vegetations can lead to bacterial endocarditis Therefore, SLE patients with valvular damage should have

antibiotic prophylaxis

Renal involvement
Prescriptions for certain antibiotics and analgesics can

adversely affect kidney function function

Therefore, the dentist should be aware of patients renal

( i.e, creatinine clearance )

Patients who are undergoing hemodialysis should receive

dental treatment on nondialysis days

Drugs commonly prescribed by dentists with predominantly kidneydependent elimination

Drugs with predominantly kidney-dependent elimination
Nonsteriodal anti-inflammatory drugs Acetylsalicylic acid Penicillins Cephalosporins Tetracycline Antifungals

Suggested adjustments
Consider increasing dose intervals and decreasing dosage Consider contacting physician if renal function is unknown

Suggested alternatives
Acetaminophen Narcotics

Drugs
certain drugs may exacerbate underlying SLE or induce a lupus

like illness
Drug-induced lupus erythematosus ( DILE ) The development of lupus-like symptoms ( commonly fever and

musculoskeletal involvement ) which resolves with cessation of offending drugs. (antihypertensive)

Procainamide (antiarrhythmics) and Hydralazine SLE exacerbation (acute lupus flare) by drugs such as penicillin,

sulfonamides and NSAIDs.

all of these drugs should be used judiciously

Dental considerations
Oral lesions Long-term corticosteroids therapy & Adrenal suppression Infection Hematologic abnormalities Cardiac diseases Renal diseases Drugs

Thank You
so much