[Multisystemic amyloidosis. Clinical study of 39 patients in Lebanon].
OBJECTIVE
Clinical analysis of multisystem amyloidosis in Lebanon, by histological type.


METHODS
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.


CONCLUSIONS
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.
Service de médecine interne, Hôtel-Dieu de France, Université Saint Joseph, Faculté de médecine, Beyrouth, Liban.
Mona
M
Saba
Aline
A
Tohmé
Gérard
G
Abadjian
Fady
F
Haddad
Elie
E
Ghayad
D000328:Adult; D000368:Aged; D000682:Amyloid / Q000032:analysis*; D017772:Amyloid Neuropathies / Q000453:epidemiology / Q000209:etiology; D000686:Amyloidosis / Q000145:classification / Q000453:epidemiology* / Q000209:etiology / Q000378:metabolism; D028226:Amyloidosis, Familial / Q000453:epidemiology; D009202:Cardiomyopathies / Q000453:epidemiology / Q000209:etiology; D015897:Comorbidity; D010505:Familial Mediterranean Fever / Q000453:epidemiology; D005260:Female; D006801:Humans; D007676:Kidney Failure, Chronic / Q000453:epidemiology / Q000209:etiology; D007861:Lebanon / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications; D009928:Organ Specificity; D011379:Prognosis; D011507:Proteinuria / Q000453:epidemiology / Q000209:etiology; D012189:Retrospective Studies; D000685:Serum Amyloid A Protein / Q000032:analysis; D014060:Tongue Diseases / Q000453:epidemiology / Q000209:etiology
D000682:Amyloid; D000685:Serum Amyloid A Protein; C035222:amyloid protein AL