Temporomandibular joint arthritis is a rare manifestation of familial Mediterranean fever and should be considered in patients of Mediterranean origin. Recently we treated four patients suffering from this condition, and intra-articularcorticosteroid injection resulted in rapid resolution of the pain and disability in two. Computed tomography confirmed the usefulness of this therapeutic modality. HEAD 81 NECK 1992;14:492-495 0 1992 John Wiley & Sons, Inc.

Familial Mediterranean fever (FMF) is a recurrent, episodic, febrile, inflammatory disorder of serosal membranes among the populations of the Mediterranean basin, eg, Arabs, Armenians, Sepharadic Jews, and Turks.lY2It may also occasionally be found in other ethnic groups. It is transmitted in the form of an autosomal recessive trait. The pathogenesis of the disease remains obscure and subject to much speculation. Various hypotheses have been proposed for etiopathogenesis of the disease. These include abnormal catecholamine metab~lism,~ autoimmune pr0cess,4~~ genetic defect in one of the families of lipocortin and proteins,6 and deficiency of C5a i n h i b i t ~ rThe . ~ C5a inhibitor plays a role in the regulation of the inflammation in serosal tissues. From the Department of Otolaryngology, Head and Neck Surgery, Soroka Medical Center, Faculty of Health Sciences. Ben-Gurion University of the Negev, Beer-Sheva, Israel

Its deficiency in FMF may explain the attacks of sterile inflammation that are characteristic of this d i ~ e a s e . ~ Synovitis in FMF usually occurs in large joints.' The temporomandibular joint (TMJ) is rarely inv~lved.~ In the protracted form, TMJ synovitis causes severe chronic pain and trismus. Residual arthritis changes may During the last 10 years we treated four cases of TMJ arthritis in FMF patients under prophylactic treatment of colchicine. In addition to discussing this rare manifestation of the disease, we report the use of intra-articular corticosteroids in the management of this condition. PATIENTS AND CLINICAL DATA

The clinical data of four FMF patients with TMJ arthritis treated in our department is tabulated in Table 1. All patients were under prophylactic treatment of colchicine at admission. In one of the patients, all consequences of the disease were observed. This, therefore, can serve as a comprehensive example. In this patient and in another one, both with a history of previous protracted TMJ arthritis, intra-articular corticosteroids were injected (3 mg bethametasone sodium phosphate and 3 mg bethametasone acetate). In both patients rapid resolution of the arthritic attack was observed; this was confirmed by computed tomography.

Address reprint requests to Dr Tovi at the Department of Otoiaryngology. Head and Neck Surgery, Soroka Medical Center, P 0 Box 151, Beer-Sheva 84101, Israel


CCC 0148-6403/92/060492- 04 0 1992 John Wiley & Sons, Inc

A 39-year-old man of Sephardic origin was referred with a 3-month history of severe pain in


Temporomandibular Arthritis in Mediterranean Fever


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Table 1. Clinical data of familial mediterraneanfever in patients with temporomandibular arthritis Age, sex

History of the disease


13, F

Multiple febrile abdominal and arthritic episodes from 10 years of age

Afebrile left synovitis


Resolved in 2 weeks


20, F

Multiple febrile abdominal and arthritic episodes from 12 years

First episode: afebrile right protracted synovitis Second episode: afebrile right synovitis

Colchicine and physiotherapy

Remained with mild trismus

Colchicine and intra-articular corticosteroid injection

Resolved in 5 days

Afebrile, left protracted synovitis and osteoporosis of the condyle First episode: afebrile left protracted synovitis with flattening of the condyle

Colchicine and physiotherapy

Resolved in 3 months

Colchicine and physiotherapy

Remained with moderate trismus

Second episode: afebrile right synovitis

Colchicine and intra-articular corticosteroid injection

Resolved in 1 week



22, M

Multiple febrile abdominal arthritic and pleural episodes from 8 years


34, M

Multiple febrile abdominal arthritic and pleural episodes; sequelae at the knee and hip from 15 years

Features of TMJ arthritis

the right facial region. He had a 15-year medical history of FMF and had been under prophylactic colchicine treatment 1 gm daily. His father and two brothers also had a history of recurrent abdominal and arthritic pain. He had suffered several episodes of febrile abdominal pain and had undergone two laparotomies for intestinal obstruction secondary to peritoneal adhesions. Two pleuritic attacks had occurred during the course of the disease. The patient had recurrent bouts of arthritis of the knee and hip. Radiographic studies had revealed narrowing of the hip joint and subchondral sclerosis. Since then he had had restricted hip movements. Recently he had developed renal failure, and amyloid deposits in the glomeruli had been detected by renal biopsy. He was first seen 8 years ago in our department because of longstanding severe left facial pain accompanied by trismus, swelling, and tenderness in the left TMJ region. A radionuclide Technetium-99 methylene diphosphonate bone scan showed an increased uptake of the isotope in the TMJ. Computed tomography (CT) demonstrated diffuse erosive changes of the left mandibular condyle. Physiotherapy and symptomatic therapy were initiated. The arthritic attack resolved within 8 months; however, narrowing of the joint and deformation of the condyle, atrophy

Temporomandibular Arthritis in Mediterranean Fever



of the left maseter muscle and a mild trismus remained as sequelae. In his second referral, the patient presented with the same signs of the disease, this time in the right TMJ. Symptomatic treatment, including transcutaneous electrical nerve stimulation, failed to control the severe pain. CT examination revealed enlargement of the right TMJ space without bony changes (Figure 1). Celestone Chronodose 1 mL (3 mg betamethasone sodium phosphate and 3 mg betamethasone acetate) were injected intra-articularly. The patient felt a dramatic decrease of pain and disability, and within a few days was symptom-free. Repeated CT confirmed the resolution of joint abnormality (Figure 2). DISCUSSION

FMF is characterized by recurrent febrile episodes of peritonitis, pleuritis, and arthritis. Although these inflammatory attacks cause much morbidity, they do not impair general health and life expectancy if amyloidosis does not occur. The onset of symptoms usually occurs in childhood and recurs at irregular intervals, abating only during pregnancy. Abdominal ain and fever are the principal clinical features! The bouts last a few days and may sometimes mimic an acute


NovernbedDecember 1992


FIGURE 1. Coronary CT of the TMJ. Note deformation of the left condyle related to the previous protracted attack of FMF arthritis and sinovitis of the right TMJ with enlargement of the joint related to the recent arthritic attack of FMF.

surgical abdomen. Pleuritic attacks may present with small effusions. Arthritis constitutes another cardinal feature of the disease and occurs in 70% of the patients.12 The most common form is mono- or oligoarthritis. Hips, knees, and shoulders are the most common sites affected.

The TMJ is a rare site of involvement.9 The arthritic attacks are self-limited and usually exhibit a short course that resolves without residual changesB8In 5% of the cases, arthritis may present with a protracted course, lasting for weeks or months.' In this condition bone damage and disability may occur. Pain, tenderness, periarticular swelling, and painful opening of the mouth are the prominent features of FMF-related TMj arthritis.lo9l1In the early stages of the arthritis, impaired joint function is usually related to the periarticular muscle spasm." Roentgenologic studies in this stage show soft tissue swelling and joint effusions enlarging the articular space. In the protracted form of arthritis, juxta-articular osteoporosis may occur. Moreover, intra-articular effusions distending the joint capsule cause stretching and narrowing of the arteries resulting in aspetic bone necrosis.' Upon resolution of the inflammation process, the erosive changes can be replaced by new bone formation, but in protracted attacks sequelae and disability may remain.lOJ1 Until the use of colchicine, FMF was untreatable. Within the past 17 years, with the daily use of this agent, the frequency of attacks was greatly reduced.l4>l5Colchicine is also important in the prevention of amyloidosis, the gravest consequence of the disease.16 It is believed that

FIGURE 2. CT of the right TMJ, before (A) and after (6) intra-articular corticosteroid injection. The latter shows complete resolution of the right TMJ synovitis.


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NovembeVDecember 1992

colchicine acts by inhibiting a variety of neutroHowever, fil functions related t o inflammati~n.'~ episodes of arthritis may still occur under colchicine treatment. Histologic features of the affected synovial tissue12i1s and analysis of the synovial effusions,12indicate a nonspecific tissue reaction. Intra-articular use of corticosteroids has been proved useful in rapid suppression of the nonspecific inflammati~n.'~ This modality of treatment is neither a specific therapy nor a cure for any kind of inflammation. It is the sole means of obtaining consistent and long lasting symptomatic relief.l9 Systemic corticosteroids have proved ineffective in FMF.12 However, intra-articular administration of this drug to two of our patients, each of whom had a previous history of protracted TMJ arthritis and sequelae, resulted in dramatic improvement of the clinical symptoms (pain and trismus). Rapid resolution of the joint lesion was confirmed by CT. Although limited, our experience with intraarticular corticosteroids suggests its usefulness in TMJ arthritis related to FMF, especially in patients with protracted arthritis, when no other therapy alleviates suffering. The suppression of the synovial inflammation may prevent irreversible articular changes and their sequelae.


Sohar E, Gafni J , Pras M, Heller H. Familial Mediterranean fever: a survey of 470 cases and review of literature. Am J Med 1967;43:227-253. Rogers DR, Shohat M, Petersen GM, Bickal J , Conglaton J, Schwabe AD, Rotter JI. Familial Mediterranean fever in Armenians: autosomal recessive inheritance with high gene frequency. Am J Med Genet 1989;34(2):168- 172. Barakat MH, Malhas LN, Gumaa KK. Catecholamine metabolism in recurrent hereditary polyserositis: Patho-

Temporomandibular Arthritis in Mediterranean Fever

genesis of acute inflammation: the retention leakage hypothesis. Biomed Pharmacother 1989;43(1):763- 769. 4 Flatau E, Shneyour A, Hadad N, Shimoni Z. Detemination by ELISA of anti-DNA antibodies in patients with familial Mediterranean fever. Zsr J Med Sci 1989;25:553-556. 5. Ben Chetrit E, Levy M: Autoantibodies in familial Mediterranean fever (recurrent polyserositis). Br J Rheumatol 1990;29:459-461. 6. Shohat M, Korenberg JR, Schwabe AD, Rotter JI. Hypothesis: familial Mediterranean fever- a genetic disorder of the lipocortin family. A m J Med Genet 1989;34(2):163- 167. 7. Ayesh SK, Ferne M, Flecner I, Babior RM, Matzner Y. Partial characterization of a CSa-inhibitor in peritoneal fluid. J Immunol 1990;144(8):3066-3070. 8. Sheh E, Pras M, Michaeli D. Protracted arthritis in familial Mediterranean fever. Rheumatol Rehabil 1977;16:102- 105. 9. Eliakim M, Levy M, Ehrenfeld M. Recurrent polyserositis. Elsevier North Holland Biomedical Press, Amsterdam, 1981, pp. 37-45. 10. Tovi F, Barmeir E, Pest M, Bar-Ziv J. Protracted temporomandibular arthritis in familial Mediterranean fever. J Oral Mmillofac Surg 1985;43:466-468. 11. Simon G. Familial Mediterranean fever with temporomandibular joint arthritis. Pediatrics 1976;57:810- 812. 12. Heller H, Gafni J , Michaeli D, et al. The arthritis of familial Mediterranean fever (FMF). Arthritis Rheum 1966;9:1- 17. 13. Shahin N, Sohar E, Dalith F. Roentgenologic findings i n familial Mediterranean disease. AJR 1960;84:269-272. 14. Dinarello CA, Wolff SM, Goldfinger SE, Dale DC, Alling DW. Colchicine therapy for familial Mediterranean fever: a double blind trial. N Engl J Med 1974;291:937-93. 15. Zemer D, Revach M, Pras M. A controlled trial of colchicine in preventing attacks of familial Mediterranean fever. N Engl J Med 1974;291:932-934. 16. Zemmer D, Pras M, Sohar E, Modan M, Cabili S, Gafni J . Colchicine in the prevention and treatment of amyloidosis of familial Mediterranean fever. N Engl J Med 1986;314:1001- 1005. 17. Gilman AG, Goodman LS, Gilman A. The pharmacological basis of therapeutics, 6th ed. New York, MacMillan, 1980;718-720. 18. Sohar E, Gafni, J , Pras M, Heller H. Familial Mediterranean fever: a survey of 470 cases and review of the literature. A m J Med 1967;43:227-253. 19. Hollander JL. Arthrocentesis technique and intrasynovial therapy. In Arthritis and allied conditions. McCarthy D (Ed.) 10th edition. Philadelphia, Lea-Febiger, 1985;541-547.


November/Decernber 1992


Temporomandibular arthritis in familial Mediterranean fever.

Temporomandibular joint arthritis is a rare manifestation of familial Mediterranean fever and should be considered in patients of Mediterranean origin...
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