periodontal manifestations in systemic sclerosis · pdf fileperiodontal manifestations in...

Continuing Education

Periodontal Manifestationsin Systemic Sclerosis

A Review

Authored by Anshul Mehra, BDS, MDS and Suneet Kumar, MBBS, MD

Course Number: 102.1

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ABOUT THE AUTHORS

Dr. Mehra is senior lecturer, Departmentof Oral Medicine and Radiology, U.P.Dental College and Research Centre,Lucknow, Uttar Pradesh, India. He can bereached at [email protected].

Dr. Kumar is sssistant professor,Department of Pathology, KasturbaMedical College, Mangalore, Karnataka,India. He can be reached [email protected].

INTRODUCTION

Scleroderma is a connective tissue disorder characterizedby tissue fibrosis, obliterative microangiopathy, and immuneabnormalities. The term scleroderma is derived from theGreek word scleros (hard) and derma (skin), hence themeaning hard skin. This term describes the pathognomonicclinical appearance of the skin seen in this disease. Theexact etiology and pathogenesis of this disease are notclear. Most commonly implicated etiological factors areenvironmental in nature (silica dust, vinyl chloride, benzene,

and tryptophan) or of viral etiology (cytomegalovirus, humanparvovirus B19). The most important predisposing factorsassociated with this disease are B cell abnormalities andgenetic susceptibility1,2 (Figure 1).

CLASSIFICATION

Scleroderma is classified as localized scleroderma andsystemic sclerosis (Figure 2). Three types of localizedscleroderma are recognized: morphea, generalizedmorphea, and linear scleroderma (en coup de sabre).Morphea is characterized by circumscribed scleroticplaques on the skin with ivory-colored centers andviolaceous borders that eventually lose hair and the abilityto sweat (Figure 3). The plaques are indurated but not


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Recommendations for Fluoride Varnish Use in Caries Management

LEARNING OBJECTIVES:

After reading this article, the individual will learn:

• the etiology, pathogenesis, clinical features, oralmanifestations, and treatment of scleroderma, and

• the periodontal manifestations seen in patients withsystemic sclerosis.

Periodontal Manifestationsin Systemic SclerosisA Review

Environmental factors?

Genetic susceptibilityaltered B cell function

Immune activationEndothelial cell

damage

Vascular damage

Multipleorgan

damage

Predisposing factors

Virus?

Fibroblast activation

Release of fibroblast growth &chemotactic factors (IL-1, IL-4,

IL-13, FGF, TGF-βNarrowing & thickening of small blood vessels

Ischemic tissue injury

Activation of T cells Activation of B cells

Activation of mast cell & macrophages

Production of autoantibodiesmainly anti-DNA topoisomerase 1

& anti-centromere antibody

Platelet activation& aggregation

Fibrosis of intimallayer

fibrosis

Release of cytokines

Release of PDGF & TNF-α

Figure 1. Pathogenesis of scleroderma.

Scleroderma

Localized scleroderma

Linear scleroderma(en coup de sabre)

Generalized morphea

Systemic sclerosis

Limited cutaneousscleroderma

(CREST syndrome)

Diffuse cutaneous

scleroderma

Morphea

Figure 2. Classification of scleroderma.

bound to the deeper structures. Most commonly, the lesionsare single or few in number, but they may be multiple. Ingeneralized morphea, there is widespread involvement ofskin with multiple indurated plaques and hyperpigmentation.Linear scleroderma is characterized by a thin band ofsclerosis that may run the entire length of an extremity, andinvolves superficial and deeper layers of the skin, withfixation to underlying structures. The lower extremities aremost often involved, followed by the upper extremities,frontal areas of the head, and anterior thorax. The lesion oflinear scleroderma of the head and face is called en coupde sabre, and these lesions may result in hemiatrophy ofthe face.

Two types of systemic sclerosis are recognized, definedby the extent of skin affected: limited cutaneous sclerodermaand diffuse cutaneous scleroderma. In limited cutaneousscleroderma, there is skin thickening in the areas solely distalto the elbows and knees, with or without facial effects. It isgenerally a milder form of disease, and the patientsfrequently have problems with digital ulcers and esophagealdysmotility. Diffuse cutaneous scleroderma has a more acuteonset and is defined by the presence of skin thickening thatis proximal, as well as distal, to the elbows and knees, withor without facial or truncal effects. There is widespreadinternal organ involvement with potentially life-threateningcardiac and renal problems1-6 (Figure 4).

CLINICAL FEATURES, TREATMENT AND PROGNOSIS

Systemic sclerosis occurs mainly in the third to fifthdecade of life. Women are affected 3 to 4 times as often asmen. The initial symptoms of systemic sclerosis are non-specific and commonly include Raynaud’s phenomenon(Figure 5); swelling or puffiness of the skin (usually on thefingers, hands, and face) that is replaced by thickening andtightening of the skin (Figure 6); ulcerations and gangreneof the fingers (Figures 7 and 8); migratory polyarthritis,flexion contractures, and sclerodactyly with digital tuftresorption, subcutaneous calcifications, joint spacenarrowing, and focal erosions of periarticular bone onradiographs (Figure 9); and less commonly, gatrointestinalproblems such as gastroesophageal reflux, dysphagia, andconstipation. The clinical course of systemic sclerosis


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Periodontal Manifestations in Systemic Sclerosis : A Review

Blood vessels reopen, causing a local “flushing” phenomenon

Exposure to cold or emotional stress

Diminished blood supply to the local tissues

Digit(s) involved turn white

Lack of oxygen

Digit(s) turn blue

Digit(s) turn red

Abnormal spasm of the blood vessels

LIMITED CUTANEOUS SSC (ISSC)• Previously known as CREST syndrome- Calcinosis cutis, Raynaud’s

phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia• Raynaud’s phenomenon for years (occasionally decades)• Skin involvement limited to hands, face, feet, and forearms (acral)• A significant (10-15%) late incidence of pulmonary hypertension, with

or without interstitial lung disease, skin calcification, telangiectasia, and gastrointestinal involvement

• High prevalence of anticentomere antibody (70-80%)

DIFFUSE CUTANEOUS SSC (dSSC)• Onset of skin changes (puffy or hidebound) within 1 year of onset of

Raynaud’s phenomenon• Truncal and acral skin involvement• Tendon friction rubs• Early and significant interstitial lung disease, renal failure• Diffuse gastrointestinal disease• Myocardial involvement• Antitopoisomerase-1 (Scl-70) antibodies (30% of patients)

Figure 4. Differences between limited cutaneous sclerodermaand diffuse cutaneous scleroderma.

Figure 3. Morphea.

Figure 5.Pathophysiology of Raynaud’sphenomenon.

varies and depends upon whether the patient developslimited or diffuse cutaneous systemic sclerosis (Figure 4).Scleroderma does not have a cure, and treatment isfocused on relief of symptoms (Figure 10). The lifespan ofthe patient with systemic sclerosis depends upon therapidity, severity, and extent of internal organ involvementand the age of the patient at the time of presentation.1,2,4-6

ORAL MANIFESTATIONS

The oral and perioral tissues are also commonlyinvolved. Most common findings include rigid lips, narroworal aperture, loss of skin folds around the mouth (mask-likeappearance), blanching of the oral mucosa due to fibrosis(Figures 11 and 12), sclerosis of the tongue (Figure 13), oraltelangiectasia, and pseudoankylosis.7-9 Oral and perioraleffects are mainly caused by skin and muscular atrophy seenin these patients. Xerostomia is also commonly seen inpatients with systemic sclerosis and is either caused byglandular fibrosis or is found to be associated with Sjogren’ssyndrome.1,4,7-11 Resorption of the mandible in areas ofmasticatory muscle attachments (angle, condyle, coronoid,and digastric) has also been reported.10,12-14

PERIODONTAL MANIFESTATIONS

The most common radiographic finding in patients withsystemic sclerosis is widening of the periodontal ligamentspace (Figure 14). This finding was first reported by Stafneand Austin15 and was later confirmed by others.7-9,16-19 Theexact mechanism for increase in the width of theperiodontal ligament is not entirely clear. The most likelyexplanation seems to be an increase in the collagensynthesis in the periodontal ligament, which is the hallmarkof this disease. Increased collagen synthesis causes anincrease in the thickness and space occupied by theperiodontal ligament. Microscopic examination also revealsexcessive collagen deposition. This increase in the volumeof the periodontal ligament exerts additional pressure onboth tooth and alveolar bone. Wood and Lee19 did notobserve any difference in the widths of the roots of theinvolved teeth between patients with systemic sclerosis andcontrols. Further, cementum is more resistant to resorption


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Figure 6. Absence of wrinkles on theforehead whenlooking upwardbecause of tighteningof the skin.

Figure 7. Swellingand puffiness ofthe nailbeds.

Figure 8. Gangreneof the finger insystemic sclerosis.

Figure 9. Hand and wrist radiographshowing digital tuftresorption andsubcutaneouscalcifications.

than bone, as bone is richly vascularizedand cementum is avascular. Therefore, thedegenerative changes caused by alteredblood flow, which is related to an increasein the pressure exerted by the periodontalligament, affect bone more readily thancementum.20 Thus this increased volumeof periodontal ligament is accommodatedat the expense of alveolar bone withoutaffecting tooth mobility.

The extent of periodontal ligamentwidening was found to be related to theseverity of the disease.4,19 Recently,trauma from occlusion has also beenreported as one of the probable causes forperiodontal ligament space widening.21 Itappears to be an unlikely cause, as themuscles of mastication undergo atrophyand thus mastication forces are reduced,thereby reducing the chances of traumafrom occlusion.10,12-14

Patients with systemic sclerosis have been reported tohave increased susceptibility to periodontal disease;however studies have shown conflicting results. Marmary,et al17 observed a significantly increased incidence ofperiodontal disease in patients with systemic sclerosis ascompared to controls, whereas Nagy, et al8 and Eversole,et al22 did not find any significant difference between casesand controls. These differences in the findings wereattributed to the differences in the control groups. Theincreased susceptibility to periodontal diseases can beexplained by both pressure ischemia and obliterativevasculopathy, which causes atrophy of the periodontalligament and, at a later stage, mobility of the involved teeth.Recent evidence also suggests that increased prevalenceof xerostomia in these patients may be one reason for anincreased prevalence of periodontitis.4,19,23

Although the mean periodontal ligament space widthwas higher in all the teeth of patients with systemicsclerosis, the increase in the widths were found to be morepronounced in the posterior teeth than in the anterior teeth.7

This can be explained by the fact that the teeth bearingmore masticatory force (ie, posterior teeth) have wider


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Figure 11. Blanchingof the oral mucosadue to fibrosis.

Figure 12. Blanchingof the ventral surfaceof the tongue withthickening of thelingual frenum.

Figure 13.Depapillation ofthe tongue due to fibrosis.

Figure 10. Symptomatic treatment for scleroderma.

SYMPTOMS TREATMENT

RAYNAUD’S Avoidance of cold exposure and smoking cessation, warm clothing, calcium PHENOMENON channel blockers, aspirin and dipyridamole, Pentoxifylline, Fluoxetine, Topical

nitroglycerine paste over digits, intravenous Alprostadil and prostaglandinSKIN FIBROSIS d-Penicillamine, Colchicine, Paraaminobenzoate, Gamma interferon,

Cyclosporine, Extracorporeal photochemotherapyGI SYMPTOMS Antacids, H2-blockers, proton-pump inhibitors, Prokinetic agents.

Reflux precautions: frequent small meals, elevation of head at end of the bed, not lying down after meals, avoidance of tea, coffee, alcohol, spicy and fatty meals

PULMONARY Systemic steroid and cyclophosphamide for pulmonary fibrosis; intravenous orDISEASE aerosolized prostacyclin for severe pulmonary hypertensionRENAL Early management of hypertension with calcium channel blockerDISEASE Management of renal failure, dialysisCARDIOVASCULAR Treatment of heart failureDISEASE Pericarditis may respond to systemic steroidsMUSCULOSKELETAL NSAIDs and paracetamol for arthralgiaSYMPTOMS Myositis may be treated with steroids, methotrexate, and azathioprineINFECTIONS Proper antibiotic therapy should be instituted

periodontal ligament spaces than those bearing less force(ie, anterior teeth).24 Thus the increase in the bulk of theperiodontal ligament in patients with systemic sclerosis isreflected more in posterior teeth as compared to theanterior teeth.

As noted, the widening of periodontal ligament spacewas typically seen in posterior teeth.15,23 Also, the incidenceof periodontal ligament space widening was found to bevariable, ranging from 10% to 37%.9,15,23,25 However, whenthe periodontal ligament space was meticulously measuredunder magnification and the mean periodontal ligamentwidth was calculated, it was found that patients withsystemic sclerosis had a higher mean width as compared tocontrols, and the mandibular lateral incisor was found to be the best tooth for predicting patients with systemicsclerosis.7,19

CONCLUSION

Patients with systemic sclerosis exhibit a generalizedpattern of widening of the periodontal ligament space, withthe average periodontal ligament width being greateraround the posterior teeth as compared to the anteriorteeth. This finding is of limited importance clinically, as theaffected teeth are not mobile initially. Also, the significanceof this finding in the diagnosis of systemic sclerosis isquestionable because prominent widening tends to occur inthe later stages of the disease.


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Figure 14. Wideningof the periodontalligament spacearound the roots ofthe posterior teeth.


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REFERENCES

1. Ciarrocca KN, Greenberg MS. Immunologic diseases. In:Greenberg MS, Glick M, eds. Burket’s Oral Medicine:Diagnosis and Treatment. 10th ed. Hamilton, Ontario,Canada: BC Decker; 2003:478-502.

2. Charles C, Clements P, Furst DE. Systemic sclerosis:hypothesis-driven treatment strategies. Lancet.2006;367:1683-1691.

3. Masi AT. Classification of systemic sclerosis (scleroderma):relationship of cutaneous subgroups in early disease tooutcome and serologic reactivity. J Rheumatol. 1988;15:894 898.

4. Gonzales TS, Coleman GC. Periodontal manifestations ofcollagen vascular disorders. Periodontol 2000. 1999;21:94-105.

5. Rowell NR, Goodfield MJ. The connective tissue diseases.In: Champion RH, Burton JL, Burns DA, Breathnach SM,eds. Rook/Wilkinson/Ebling Textbook of Dermatology. 6thed. Oxford, England: Blackwell Science; 1998: 2437-2575.

6. Tu JH, Eisen AZ. Scleroderma. In: Freedberg IM, Eisen AZ,Wolff K, Austen KF, Goldsmith LA, Katz S, eds. Fitzpatrick’sDermatology in General Medicine. 5th ed. New York, NY:McGraw-Hill; 1999:2023-2033.

7. Alexandridis C, White SC. Periodontal ligament changes inpatients with progressive systemic sclerosis. Oral Surg OralMed Oral Pathol. 1984;58:113-118.

8. Nagy G, Kovács J, Zeher M, et al. Analysis of the oralmanifestations of systemic sclerosis. Oral Surg Oral MedOral Pathol. 1994;77:141-146.

9. Rout PG, Hamburger J, Potts AJ. Orofacial radiologicalmanifestations of systemic sclerosis. DentomaxillofacRadiol. 1996;25:193-196.

10. Haers PE, Sailer HF. Mandibular resorption due to systemicsclerosis. Case report of surgical correction of a secondaryopen bite de-formity. Int J Oral Maxillofac Surg. 1995;24:261-267.

11. Avouac J, Sordet C, Depinay C, et al. Systemic sclerosis-associated Sjogren’s syndrome and relationship to thelimited cutaneous subtype: results of a prospective study of sicca syndrome in 133 consecutive patients. ArthritisRheum. 2006;54:2243-2249.

12. Rubin MM, Sanfilippo RJ. Resorption of the mandibularangle in progressive systemic sclerosis: case report. J Oral Maxillofac Surg. 1992;50:75-77.

13. Ramon Y, Samra H, Oberman M. Mandibular condylosis andapertognathia as presenting symptoms in progressivesystemic sclerosis (scleroderma). Pattern of mandibularbony lesions and atrophy of masticatory muscles in PSS,presumably caused by affected muscular arteries. Oral SurgOral Med Oral Pathol. 1987;63:269-274.

14. Auluck A, Pai KM, Shetty C, et al. Mandibular resorption inprogressive systemic sclerosis: a report of three cases.Dentomaxillofac Radiol. 2005;34:384-386.

15. Stafne EC, Austin LT. A characteristic dental finding inpatients with acrosclerosis and diffuse scleroderma. Am J Orthod Oral Surg. 1944;30:25.

16. Rowell NR, Hopper FE. The periodontal membrane insystemic sclerosis. Br J Dermatol. 1977;96:15-20.

17. Marmary Y, Glaiss R, Pisanty S. Scleroderma: oralmanifestations. Oral Surg Oral Med Oral Pathol. 1981;52:32-37.

18. Robbins JW, Craig RM Jr, Correll RW. Symmetrical widening ofthe periodontal ligament space in a patient with multiplesystemic problems. J Am Dent Assoc. 1986;113:307-308.

19. Wood RE, Lee P. Analysis of the oral manifestations ofsystemic sclerosis (scleroderma). Oral Surg Oral Med OralPathol. 1988;65:172-178.

20. Bhaskar SN, ed. Orban’s Oral Histology and Embryology.11th ed. St Louis, MO: Mosby; 1990:180-202.

21. Auluck A. Widening of periodontal ligament space andmandibular resorption in patients with systemic sclerosis.Dentomaxillofac Radiol. 2007;36:441-442.

22. Eversole LR, Jacobsen PL, Stone CE. Oral and gingivalchanges in systemic sclerosis (scleroderma). J Periodontol.1984;55:175-178.

23. Gores RJ. Dental characteristics associated withacrosclerosis and diffuse scleroderma. J Am Dent Assoc.1957;54:755-759.

24. Coolidge ED. The thickness of human periodontalmembrane. J Am Dent Assoc and Dent Cosmos.1937;24:1260-1270.

25. White SC, Frey NW, Blaschke DD, et al. Oral radiographicchanges in patients with progressive systemic sclerosis(scleroderma). J Am Dent Assoc. 1977;94:1178-1182.

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POST EXAMINATION QUESTIONS

1. Scleroderma means ____.

a. hard skin

b. soft skin

c. elastic skin

d. fragile skin

2. Circumscribed sclerotic plaques with ivory-coloredcenters and violaceous borders that eventually losehair and the ability tosweat are seen in ____.

a. morphea

b. linear scleroderma

c. limited cutaneous scleroderma

d. diffuse cutaneous scleroderma

3. En coup de sabre is the linear sclerodermainvolving the ____.

a. upper extremities

b. lower extremities

c. thorax

d. head and face

4. Raynaud’s phenomenon is ____.

a. an abnormal spasm of blood vessels

b. an abnormal spasm of muscles

c. damage of the nerves due to fibrosis

d. none of the above

5. The sequence of change in skin color seen inRaynaud’s phenomenon is ____.

a. white, blue, red

b. red, blue, white

c. white, blue, pink

d. blue, white, red

6. The characteristic appearance of the face seen inpatients with scleroderma is ____.

a. bird-like appearance

b. fish-like appearance

c. mask-like appearance

d. simian-like appearance

7. The most common periodontal finding seen inpatients with scleroderma is ____.

a. widening of periodontal ligament space involving the entire root, with mobility of the tooth

b. widening of periodontal ligament space involving the entire root, without mobility of tooth

c. widening of periodontal ligament space involving the apex only

d. generalized loss of lamina dura

8. Widening of periodontal ligament space occurs at theexpense of bone and not the tooth because ____.

a. cementum is avascular

b. cementum is more resistant to resorption than bone

c. bone is richly vascularized, so the degenerative changes caused by interference in the blood circulation affect bone more easily than cementum

d. all of the above


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