Respiratory muscle weakness and ventilatory failure in AL amyloidosis with muscular pseudohypertrophy

Rafael M. Santiago, David Scharnhorst, Gary Ratkin, Edmond C. Crouch

Research output: Contribution to journalArticle

23 Scopus citations

Abstract

Generalized muscle weakness culminating in ventilatory failure developed in a 59-year-old man with kappa light chain multiple myeloma. Physical examination demonstrated skeletal muscle enlargement, severe proximal muscle weakness, and macroglossia, consistent with amyloid-associated muscle pseudohypertrophy. Pulmonary function studies revealed a severe restrictive abnormality with a low maximal inspiratory pressure and maximal voluntary ventilation. Arterial blood gas values and chest radiographic results were normal. There was no clinical evidence of cardiac or central nervous system disease. At autopsy, skeletal muscles and diaphragm were diffusely infiltrated by amyloid. There was also multifocal deposition of amyloid in alveolar septae, esophagus, and subendocardium. This report suggests that ventilatory failure may occur as a complication of myeloma-associated (AL) amyloidosis involving the respiratory muscles.

Original languageEnglish
Pages (from-to)175-178
Number of pages4
JournalThe American journal of medicine
Volume83
Issue number1
DOIs
StatePublished - Jul 1987

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