Elie Ghayad

Amyloses multisystémiques: Étude clinique de 39 patients au Liban

Resume Objectif Analyse clinique des amyloses generalisees au Liban, classees selon le type histologique. Methode Etude retrospective de 39 cas d’amylose multi-systemique, diagnostiques histologiquement dans un centre hospitalier universitaire entre 1991 et 2002. Les donnees cliniques analysees etaient l’âge, le sexe, le mode de presentation, le delai entre les premiers symptomes et le diagnostic, les diverses manifestations cliniques, les maladies associees, une histoire familiale d’amylose, le(s) site(s) de la biopsie, la presence d’une gammapathie monoclonale serique ou urinaire, le type immunohistochimique, le pronostic et le traitement. Resultats L’âge moyen lors du diagnostic etait de 56 ± 18 ans avec un sex ratio (H/F) de 1,4. Cinquante-quatre pour cent (21/39) etaient des amyloses AL ( amyloid light chain ), 36 % (14/39) des amyloses AA ( amyloid associated ) et 10 % (4/39) n’ont pas ete typees. Parmi les 21 amyloses AL, 12 etaient isolees (57 %) et 9 (43 %) associees au myelome multiple. Sur les 14 amyloses AA, 7 etaient associees a une fievre mediterraneenne familiale et 5 a des infections ou inflammations chroniques. Le mode de debut le plus frequent etait renal. Dans les amyloses AL avec myelome, les symptomes initiaux etaient plutot dus a l’amylose et non au myelome. L’atteinte renale etait observee dans 95 % (37/39) des cas (95 % des amyloses AL versus 93 % des amyloses AA); elle s’etait manifestee par une proteinurie dans 87 % des cas et une insuffisance renale dans 72 % des cas. L’atteinte cardiaque (57 % des amyloses AL versus 7 % des amyloses AA; p > 0,05), la macroglossie (19 % des amyloses AL versus 0 % des amyloses AA; p > 0,05) et l’atteinte neurologique (24 % des AL versus 7 % des AA; p > 0,05) sont plus frequentes dans l’amylose AL. Nous avons eu 7 deces (18 %), tous dans le type AL (5 au cours d’un myelome). L’insuffisance cardiaque est la cause de mort par amylose la plus frequente. Conclusion Les amyloses multisystemiques sont frequentes au Liban et la fievre mediterraneenne familiale reste une cause frequente d’amylose secondaire. Une classification exacte du type d’amylose est essentielle pour le pronostic et le traitement. L’amylose AL, particulierement celle qui s’associe au myelome, et l’atteinte cardiaque sont des elements de mauvais pronostic.OBJECTIVE Clinical analysis of multisystem amyloidosis in Lebanon, by histological type. METHOD Retrospective study of 39 cases of multisystem amyloidosis diagnosed histologically in a university hospital center between 1991 and 2002. It analyzed the following clinical data: age, gender, type of presentation, time from symptom onset to diagnosis, clinical features, concomitant diseases, family history of amyloidosis, biopsy sites, presence of urinary or serum monoclonal gammopathy, immunohistochemical type, prognosis and treatment. RESULTS Median age at diagnosis was 56+/-18 years. The overall ratio of men to woman was 1.4. AL amyloidosis (amyloid light chain) accounted for 54% (21/39) of the cases, AA (amyloid-associated) amyloidosis 36% (14/39), while 10% (4/39) were not typed. Among the 21 cases of AL amyloidosis, 12 were idiopathic (57%) and 9 (43%) were associated with multiple myeloma; among the 14 cases of AA amyloidosis, 7 were associated with familial Mediterranean fever and 5 with chronic disorders. Proteinuria was often the first symptom. The initial manifestations in AL amyloidosis patients with myeloma were more often related to amyloidosis than to myeloma. Renal involvement was seen in 95% (37/39) of all cases (95% of AL versus 93% of AA), proteinuria in 87% of cases and renal failure in 72%. Cardiac amyloidosis (57% of AL versus 7% of AA; p>0.05), infiltration of the tongue (19% of AL versus 0% of AA; p>0.05) and neurological manifestations (24% of AL versus 7% of AA; p>0.05) were more frequent in AL amyloidosis. The 7 patients who died (18%) had AL amyloidosis (5 of them had myeloma). Heart failure was the most frequent cause of death related to amyloid. CONCLUSION Multisystem amyloidosis is frequent in Lebanon and familial Mediterranean fever is still frequently associated with the secondary type. Accurate diagnosis and classification are essential for the prognosis and treatment of the disease. Poor prognosis was associated with the AL type, especially when accompanied by myeloma, and with cardiac amyloidosis.