Q J Med 2003; 96:171–173 doi:10.1093/qjmed/hcg022
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QJM Type 1 autoimmune hepatitis revealed by a dysphonia related to cricoarytenoid arthritis Sir, Autoimmune hepatitis disease may be accompanied, or revealed by, extra-hepatic (particularly rheumatological) manifestations, in approximately 5% of patients.1 These manifestations include arthralgia, symmetrical non-erosive, non-deforming polyarthritis, commonly localized on large articulations (knees, elbows, ankles and wrists), and myalgia.1 We report a case of type 1 autoimmune hepatitis revealed by a dysphonia related to cricoarytenoid arthritis, which has not previously been described to our knowledge. A 45-year-old woman presented with a sudden dysphonia accompanied by dysaesthesiae of the upper right limb. Fifteen days earlier, she had been seen for a 38 8C fever, oligoarthralgia, inflammatory syndrome (CRP )100 mg/l, VS )100 mm) and an acute cytolysis (ASAT and ALAT 4–53normal), which regressed spontaneously. General clinical examination was normal, except that examination of the upper respiratory tract revealed a reduced mobility of the vocal cords related to probable cricoarytenoid arthritis. Standard laboratory tests showed evidence of inflammation (CRP 50 mg/l, VS 80 mm), and only mild acute cytolysis (ASAT and ALAT 1.5–23normal). Serological tests for hepatitis A, B and C viruses, Epstein-Barr virus, cytomegalovirus, human immunodeficiency virus (HIV), B19 Parvovirus, Coxiella burnetti, Bartonella sp., Rickettsia sp., Borrelia burgdorferi and Francisella tularensis were negative, as were tests for tuberculous infection. High titres of anti-smooth muscle (anti-actin) antibodies (1:200 wnormal 1:20x, by enzyme-linked immunoassay) were detected, suggesting an type 1 autoimmune hepatitis. Anti-DNA, anti-LKM1, anti-hepatic cytosol, anti-mitochondrial antibodies, rheumatoid factor and angiotensin-converting enzyme were absent. Only an oligoclonal profile with a monoclonal IgG lambda was detected. Hepatic biopsy confirmed the diagnosis of early type 1 autoimmune hepatitis. Electromyograms of the limb and of the vocal cords were normal, as was the cerebral RMN and analysis of the cerebral fluid. ß Association of Physicians 2003
Thus, a diagnosis of dysphonia arising from cricoarytenoid arthritis, related to in type 1 autoimmune hepatitis, was made. A short period of corticosteroid therapy (prednisolone 40 mg/day for 7 days, then 20 mg/day for 7 days) with orthophonic rehabilitation produced regression of the dysphonia and normalization of the biological parameters. No relapse was seen one year later, when serum anti-smooth muscles antibodies remained at 1:100. We believe this to be the first reported case of type 1 autoimmune hepatitis revealed by cricoarytenoid arthritis. To our knowledge, only few systemic diseases have been described with cricoarytenoid arthritis:2 rheumatoid polyarthritis,3 acute systemic lupus erythematosus4 and ankylosing spondylitis.5 In our case, the diagnosis of type 1 autoimmune hepatitis was well established by high titres of anti-smooth muscle and histological data (hepatic biopsy). As the usual aetiologies of cricoarytenoid arthritis were not found,2 this manifestation was probably related to the type 1 autoimmune hepatitis, by temporal arguments. We therefore propose that dysphonia related to cricoarytenoid arthritis be considered as a possible cause of autoimmune hepatitis. N.H. Loukili C. Pettorin E. Noel E. Andre`s Department of Internal Medicine Hoˆ pitaux Universitaires de Strasbourg France e-mail:
[email protected]
References 1. Ben-Ari Z, Czaja AJ. Autoimmune hepatitis and its variant syndromes. Gut 2001; 49:589–94. 2. Loehrl TA, Smith TL. Inflammatory and granulomatous lesions of the larynx and pharynx. Am J Med 2001; 111(Suppl. 8A):113–17S. 3. Absalom AR, Watts R, Kong A. Airway obstruction caused by rheumatoid cricoarytenoid arthritis. Lancet 1998; 351:1099–100.
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4. Karim A, Ahmed S, Siddiqui R, Marder GS, Mattana J. Severe upper airway obstruction from cricoarytenoiditis as the sole presenting manifestation of a systemic lupus erythematosus flare. Chest 2002; 121:990–3. 5. Miller FR, Wanamaker JR, Hicks DM, Tucker HM. Cricoarytenoid arthritis and ankylosing spondylitis. Arch Otolaryngol Head Neck Surg 1994; 120:214–16.
