Symptomatic macroglossia and tongue myositis in polymyositisTreatment with corticosteroids and intravenous immunoglobulin.код для вставкиСкачать
ARTHRITIS & RHEUMATISM Vol. 46, No. 10, October 2002, pp 2762–2764 DOI 10.1002/art.10548 © 2002, American College of Rheumatology Symptomatic Macroglossia and Tongue Myositis in Polymyositis Treatment With Corticosteroids and Intravenous Immunoglobulin E. Chauvet, L. Sailler, M. Carreiro, J. R. Paoli, P. Arrue, L. Astudillo, F. Oksmann, M. B. Delisle, and Ph. Arlet Symptomatic macroglossia occurs in some rare congenital muscle diseases, such as Becker’s and Duchenne’s dystrophies or Pompe’s disease. Herein we describe a case of symptomatic macroglossia with myositis of the tongue occurring in a patient with polymyositis. Tongue myositis was evidenced by dysarthria, frequent biting during mastication, swallowing difficulties without aspiration, and noisy breathing. Magnetic resonance imaging showed homogeneous hypertrophy of the tongue, especially the mouth’s floor muscles. The diagnosis of tongue myositis was established by electromyography and biopsy. No other cause for the macroglossia was found. Symptoms resolved quickly with corticosteroid and intravenous immunoglobulin treatment. To our knowledge, this is the first reported case of symptomatic tongue myositis occurring in the course of polymyositis. CASE REPORT The patient was referred for consultation in March 1999, at the age of 58 years, for fatigability due to muscular weakness involving the shoulder and pelvic girdles and the limbs. The symptoms had been worsening for 2 years. She was obese (height 1.60 meters, weight 97 kg) and had diabetes mellitus, chronic obstructive pulmonary disease, and hypertension. Her treatment included insulin, captopril, and bambuterol. On physical examination, a slight decrease in muscle strength in all 4 limbs and girdles was noted. She had pain on muscle pressure. No skin abnormalities were observed. The patient reported that her tongue had started to become abnormally large 6–12 months previously. The erythrocyte sedimentation rate was 4 mm/ hour. The creatinine phosphokinase (CPK) level was elevated (2,086 IU; normal ⬍135). Antinuclear antibodies were weakly positive (1:80) by immunofluorescence (IF) on rat liver, and negative by IF on HEp-2 cells. SSA, SSB, RNP, PM-Scl, Mi-2, signal recognition particle, and Jo-1 autoantibodies were not detected by immunodiffusion and immunoblot. Electromyography confirmed a diffuse typical myositic process. Muscle biopsy revealed necrotic muscle fibers, regenerating fibers, and an endo- and perimysial inflammatory infiltrate. Immunohistochemical analyses for dystrophins 1, 2, and 3 showed no deficiency. Electron microscopy revealed no specific ultrastructural abnormality. The final diagnosis was polymyositis meeting the Bohan and Peter criteria (5). Treatment with methotrexate (10 mg/week) was instituted. Fatigability improved slowly. In October 1999, the patient reported progressive dysarthria, frequent tongue-biting during mastication, dysphagia, and noisy breathing. On physical examination, macroglossia was noted (Figure 1) and the submental region appeared indurated, as if the muscles of the region were hypertro- Tongue myositis has been described to occur rarely in the course of some inflammatory muscle diseases (1–4), but has not been reported in association with polymyositis. Herein we describe the case of a patient with polymyositis in whom macroglossia and symptomatic tongue myositis developed. These symptoms were treated successfully with corticosteroids and intravenous immunoglobulin (IVIG). E. Chauvet, MD, L. Sailler, MD, M. Carreiro, MD, J. R. Paoli, MD, P. Arrue, MD, L. Astudillo, MD, F. Oksmann, MD, M. B. Delisle, MD, Ph. Arlet, MD: Centre Hospitalier Universitaire Rangueil-Larrey, Toulouse Cedex, France. Address correspondence and reprint requests to L. Sailler, MD, Service de Médecine Interne, CHU Rangueil-Larrey, 24 Chemin de Pouvourville, 31403 Toulouse Cedex, France. E-mail: firstname.lastname@example.org. Submitted for publication February 8, 2002; accepted in revised form June 18, 2002. 2762 TONGUE MYOSITIS ASSOCIATED WITH POLYMYOSITIS 2763 Figure 2. Magnetic resonance imaging of the tongue and oropharynx (T1-weighted sequence). 1, Muscle hypertrophy in the floor of the mouth. 2, Disappearance of the tongue vallecula. 3, Narrowing of the upper airways. Figure 1. Macroglossia in the patient. phic. There was a slight deficit in proximal and distal muscle strength. The CPK level was 1,642 IU. Blood ␣-glucosidase activity was normal. Captopril-induced angioedema was suspected, and the captopril treatment was stopped and prednisone treatment started at an initial dosage of 0.7 mg/kg/day (70 mg/day) in November 1999. The oropharyngeal symptoms improved greatly within a few days but the diabetes became difficult to control, so prednisone was stopped in January 2000. At this time, the CPK level was 194 IU. The oropharyngeal symptoms worsened shortly after discontinuation of the corticosteroid treatment, and prednisone was reinstituted at a moderately effective dosage of 20 mg/day. In February 2000, computed tomography scanning and magnetic resonance imaging (MRI) (T1weighted, T2-weighted, and fat-suppressed sequences) of the oropharynx revealed the presence of a general and homogeneous hypertrophy of the tongue and of its constrictive muscles, particularly the genioglossus, with a strong attenuation of the tongue vallecula (Figure 2). The upper airways were narrowed. There was no evidence of edematous or lipomatous infiltration. Hor- mone levels (thyrotropin, growth hormone, and insulin growth factor 1) were within normal ranges. Electromyography of the tongue and submental muscles revealed signs of a diffuse myogenic process. Tongue biopsy was performed and showed a multifocal inflammatory infiltrate with septal and endomysial fibrosis (Figure 3). Congo red staining of tongue, muscle, bone, salivary gland, and skin biopsy specimens showed no evidence of amyloidosis. IVIG was given for 2 days (2 gm/kg/day). The oropharyngeal symptoms resolved in the week following the IVIG infusions, but recurred 3 weeks later. Six Figure 3. Specimen from a tongue biopsy, showing 1, a multifocal inflammatory infiltrate and 2, septal and endomysial fibrosis. 2764 CHAUVET ET AL monthly infusions were prescribed and showed persistent efficacy, and corticosteroid treatment was gradually tapered. Since November 2000, the patient has been treated with azathioprine and low-dose prednisone (7.5 mg/day in April 2002). She feels well. The macroglossia persists, but with no associated symptoms. DISCUSSION We have described the case of a patient who presented with polymyositis, symptomatic macroglossia, and tongue myositis. The diagnosis of polymyositis was certain according to the Bohan and Peter criteria (5). The most common causes of macroglossia (amyloidosis, myxedema, acromegaly) were ruled out, and tongue angioedema due to captopril (6) was excluded after this treatment was stopped without any beneficial effect. Both tongue electromyography and tongue biopsy confirmed the myositic process, and macroglossia-related symptoms were clearly responsive to corticosteroids initially, and then to intravenous immunoglobulin. Macroglossia in association with muscle diseases is rare. It has been described to occur in some genetic disorders (Duchenne’s and Becker’s dystrophies, Pompe’s disease, and C283Y mutation sarcoglycanopathy) (7–9). Duchenne’s and Becker’s dystrophies were excluded in our patient since we did not find any dystrophin abnormality. Blood ␣-glucosidase activity was normal, and neither MRI nor biopsy showed the fatty and fibrous involution of the tongue characteristic of Pompe’s disease. C283Y mutation is a sarcoglycanopathy occurring exclusively in young gypsies (9). Another condition to be considered in the differential diagnosis is carcinoma of the tongue, which can simulate focal tongue myositis (10,11) or cause dermatomyositis (12). Therefore, the need for muscle biopsy must be emphasized in patients presenting with inflammatory muscle disease and suspected to have tongue myositis, in order to exclude the possibility of a neoplastic disorder. In our patient, there was a question as to whether the macroglossia was due solely to polymyositis. She had never noted macroglossia before the disease onset. At an early stage, polymyositis sometimes results in muscle hypertrophy (1), and muscle hypertrophy in the floor of the mouth was revealed by the MRI in this case. Imaging showed no signal suggesting muscle inflammation, however, perhaps because the patient had been taking prednisone at 20 mg/day for 2 weeks at that time. In addition, the symptoms improved very quickly with corticosteroid and IVIG treatment. However, the tongue volume did not decrease. Therefore, the oropharyngeal symptoms in this patient were probably due mainly to the hypomotility of the tongue caused by the myositic process, and not to the macroglossia itself. REFERENCES 1. Chérin P, Herson S. Myopathies inflammatoires. In: Kahn MF, editor. Maladies et syndromes systémiques. 4th ed. Paris: Médecine-Sciences Flammarion; 2000. p. 597–622. 2. Khan AR, Bertorini TE, Horner LH. Tongue involvement in a patient with granulomatous myositis. South Med J 1997;90:937–9. 3. Sanger RG, Kirby JW. The oral and facial manifestations of dermatomyositis with calcinosis: report of a case. Oral Surg Oral Med Oral Pathol 1973;35:476–88. 4. Bamanikar S, Mathew M. 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