Type 1 autoimmune hepatitis revealed by a dysphonia related to cricoarytenoid arthritis.

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 arth-ralgia, symmetrical non-erosive, non-deforming poly-arthritis, 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). 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 immuno-assay) were detected, suggesting an type 1 autoimmune hepatitis. Anti-DNA, anti-LKM1, anti-hepatic cyto-sol, 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. Thus, a diagnosis of dysphonia arising from cricoarytenoid arthritis, related to in type 1 auto-immune 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 crico-arytenoid arthritis. To our knowledge, only few systemic diseases have been described with cricoarytenoid arthritis: 2 rheumatoid polyarthritis, 3 acute systemic lupus erythematosus 4 and ankylo-sing 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 …