Scleroderma and the Temporomandibular Joint: Reconstruction in 2 VariantsJournal of Oral and Maxillofacial Surgery


Robert Bruce MacIntosh, Prasanna-Kumar Shivapuja, Rabia Naqvi
Otorhinolaryngology / Oral Surgery / Surgery


Scleroderma and the Temporomandibular

Joint: Reconstruction in 2 Variants

Robert Bruce MacIntosh, DDS,* Prasanna-Kumar Shivapuja, BDS, MDS, DDS, MS,y and Rabia Naqvi, BSz

Purpose: This article reviews the pathophysiology of scleroderma (systemic sclerosis [SSc]) and its the t ith ent d epre he v ell an esp ely 9 show that surg lofac rosis (SSc), an autoimmune connective tissue disorder of im ease in th in th surv nez sive

T ately ized

CRE nom angi (dcS the dcSSc has only a 15% survival rate at that point. the tifice ucral ory her nd he nd tes ith ing 7-10 nty *Clinica




UDM School of Dentistry, 2700 Martin Luther King Boulevard,

Detroit, MI 48208-2576; e-mail: Mac999@bfomfs.comyConsultant in Orthodontics, Oral and Maxillofacial Surgery vice, St John Health System, Detroit, MI. zSenior Student, University of Detroit Mercy School of Dentistry, troit, MI.

Address correspondence and reprint requests to Dr MacIntosh:  2015 American Association of Oral and Maxillofacial Surgeons 0278-2391/14/01818-7 Professor, Department of Oral and Maxillofacial Surgery, of Detroit Mercy School of Dentistry, Detroit, MI.

Received August 31 2014

Accepted December 6 2014perfectly defined origin. It is a heterogenous discharacterized by excessive deposition of collagen e skin and internal organs, with severe alterations e microvasculature. Krieg and Meurer1 provided a ey of the disease a quarter century ago, and Jimeand Derk2 provided a more recent comprehenoverview. wo subtypes of the disease exist and are immedirecognizable by the sclerotic skin changes. Localcutaneous SSc (lcSSc), previously identified as the

ST syndrome (soft tissue calcinosis, Raynaud pheenon, esophageal dystonia, sclerodactyly, and telectasias) is more limited; diffuse cutaneous SSc

Sc) involves multiple organ systems in addition to characteristic fibrotic skin presentation. LcSSc has

Three major processes appear to be involved in pathologic progression of SSc. Immunologically, an broblast antibodies act to alter fibroblasts to produ destructive cytokines that provoke excessive prod tion of collagen and progressive cutaneous and visce fibrosis.2-6 In addition, an exaggerated inflammat response, evoked through T and B cells and ot monocytes, leads to the activation of fibroblasts a the binding of noxious agents to endothelial cells. T third element, the proliferation of endothelial cells a subendothelial deposition of collagen, precipita narrowing and thrombosis of vessel lumina w consequent altered endothelial permeability, lead to hypoxia, ischemia, and vascular occlusion.4,5,

Several investigators have emphasized the uncertaidestructive effects on the mandible in general and cusses the considerations of operating on patients w

TMJ reconstruction in patients with the diagnosis.

Patients andMethods: Two patients with differ struction with costochondral grafts. The patients r disease and different outcomes as determined by t

Results: The 2 patients tolerated the surgeries w term. One patient thrives and continues to dowell d the second patient died of her disease approximat

Conclusions: The experience with these 2 cases reconstruction with anticipated good results, but mount in determining the feasibility of corrective  2015 American Association of Oral and Maxil

J Oral Maxillofac Surg 73:1199-1210, 2015

Scleroderma is a descriptive term for systemic scle-1199emporomandibular joint (TMJ) in particular. It disdevastating chronic disease and presents 2 cases of egrees of SSc involvement underwent TMJ reconsent the surgical considerations pertinent to this ariance in severity of their afflictions. d exhibited improvement in function in the longite her SSc approximately 10 years postoperatively; years after her initial surgical care. ed that patients with SSc can safely undergo TMJ the overall severity of the disease remains paraery under this diagnosis. ial Surgeons a 50% survival rate 12 years after diagnosis, whereas 3 1200 SCLERODERMA AND THE TEMPOROMANDIBULAR JOINTas to how these 3 pathologic processes link together to provoke the progressive fibrotic process.2,3,5,6

Several investigators have discussed genetic etiologic influences, but the general consensus is that environmental factors play the greater role in the genesis of

SSc.5,6,11 Environmental agents, such as vinyl chloride and potentially noxious inhalants to which miners are exposed, have been implicated in the etiology.

However, most suspicion falls on infectious sources, with herpes viruses, retroviruses, and human cytomegalovirus being the most often implicated.

Some investigators have suggested that autoantibodies are produced as a result of ‘‘molecular mimicry,’’ with the suggestion that antibodies against self-antigens are formed because of the antigens resembling viral or bacterial proteins.2,6,9,11 Anticentromere antibodies (ACAs) are more common in lcSSc; anti-topoisomerase antibodies (ATAs) are more common in dcSSc and signal a more unfavorable prognosis.

The overall prevalence of SSc is reported to be 242 to 286 per million in the United States, and the annual incidence is 9 to 19 per million per year.2,11,12 The disease is more common in women, particularly those 30 to 50 years of age, but the prognosis is worse for men.8,12 SSc appears to be more common in blacks than whites and to be more severe. Black

Americans have higher titers of ATA-1 (anti-Scl-70), and white Americans have higher levels of ACAs and a better prognosis. In clinical terms, this means that

American blacks are more likely to be afflicted with dcSSc and American whites are more likely to be afflicted with lcSSc.2,13-15

The universal feature of SSc, whether lcSSc or dcSSc, is thickening and fibrosis of the skin. This feature can be earlier in onset and of longer duration in limited SSc, but characteristically progresses more rapidly, and suggests earlier systemic involvement inpatientswithdcSSc. The fibrosis can involve any skin surface, but is less common on the back andbuttocks.3,16 The secondmost clinically apparent and often initial manifestation of the disease is the Raynaud phenomenon; a shorter period of the