Macroglossia como primeira manifestação clínica da amiloidose primária

Abstract

Amyloidosis is a rare disease caused by pathological deposit of an amyloid extracellular proteinaceus material. We report a case of a 50-year-old man with history of lips and tongue swelling associated to periorbital ecchimosys for 6 months. At the physical examination an important macroglossia was observed. Complementary tests were accomplished, and the pathological examination of the tongue biopsy confirmed the diagnosis. The patient was treated with prednisone and melphalan with an unsatisfactory response. Along the follow up the patient developed heart failure, and died 7 months after diagnosis of pulmonary infection and sepsis.

Dysphagia due to macroglossia in a patient with amyloidosis associated with multiple myeloma: A case report

“…It is a rare disease and it can be difficult to diagnose. The first clinical manifestations may be nonspecific, and diagnosis is usually confirmed after a particular body organ has been affected . Specific symptoms may depend on the organ involved …”

“…The first clinical manifestations may be nonspecific, and diagnosis is usually confirmed after a particular body organ has been affected. 2 Specific symptoms may depend on the organ involved. 3 Systemic amyloidosis may be primary and it can occur in association with multiple myeloma, and secondary as a result of hemodialysis and heredofamilial disorders.…”

Macroglossia and periorbital ecchymoses in a patient with systemic amyloidosis: A case report

“…Amyloidosis is a rare disease and a diagnostic challenge because of its nonspecific presenting features [1,2].…”

Terapia com anti-TNF na amiloidose renal em artrite reumatoide refratária: uma nova perspectiva terapêutica

“…Amyloidosis AL and AA represent approximately 95% of the cases. 3 Amyloidosis AA, secondary to chronic inflammation, is the most common type of systemic amyloidosis. 1 It represents an important and rare complication of rheumatoid arthritis (RA) caused by deposits of amyloid originated from acute-phase proteins.…”

“…Due to the possibility of controlling the inflammatory activity, anti-TNF agents represent and alternative treatment of secondary types. 6,7 The Brazilian Journal of Rheumatology has published three cases of amyloidosis, two on primary amyloidosis 3,8 and one secondary to hemodialysis, 9 over the last ten years. The objective of this report was to illustrate secondary renal amyloidosis as a rare complication of RA, and to discuss the use of anti-TNFα (etanercept) agents as a new therapeutic perspective.…”