2008 ICD-9-CM Volume 1 Diagnosis Codes Home > Endocrine, Nutritional And Metabolic Diseases, And Immunity Disorders 240-279 > Other Metabolic Disorders And Immunity Disorders 270-279 > Other And Unspecified Disorders Of Metabolism 277.* > 2008 ICD-9-CM Diagnosis 277.3 Amyloidosis  On October 1, 2008 the 2009 ICD-9-CM codes came into effect. Therefore, this code may be out of date. The 2009 version of ICD-9-CM 277.3 can be accessed here. A disease characterised by the deposition of amyloid in various organs of the body. It is generally considered that the amyloidoses share common pathogenic mechanisms which lead to protein fibril formation and deposition. These mechanisms may include structural, metabolic, genetic and environmental factors that may be common to all forms of amyloidosis. Primary amyloidosis, while the most common form of amyloidosis, is a sporadic disease and, therefore, can only be investigated on a case by case basis. Secondary (reactive) amyloidosis occurs in subjects with chronic inflammatory disease or cancer but factors which predict amyloid formation and which subject is going to be affected, are not readily apparent. Alzheimer disease, while very common in the aged population, is unpredictable except for those rare forms of familial Alzheimer disease with characterized mutations in specific genes. Hereditary transthyretin amyloidosis has generally been considered a rare disease and the original impetus to study the pathogenesis of this disease was because it represented a human model of systemic amyloidosis. A group of diseases in which protein is deposited in specific organs (localized amyloidosis) or throughout the body (systemic amyloidosis). Amyloidosis may be either primary (with no known cause) or secondary (caused by another disease, including some types of cancer). Generally, primary amyloidosis affects the nerves, skin, tongue, joints, heart, and liver; secondary amyloidosis often affects the spleen, kidneys, liver, and adrenal glands. A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits. An autosomal recessive inherited disease characterized by episodic FEVER; severe ABDOMINAL PAIN; PLEURISY; ARTHRITIS; and a characteristic ankle RASH. Disease flare-ups tend to last 3 days, and individuals are asymptomatic between attacks. ORCHITIS; benign recurrent MENINGITIS; HEADACHE; and AMYLOID nephropathy may also occur. (From Medicine (Baltimore) 1998 Jul;77(4):268-97) 277.3 is a non-specific code that cannot be used to specify a diagnosis View the ICD-9-CM Volume 1 277.* hierarchy ICD9Data's sister site ProcedureCodes.com launches with the 2008/09 CPT® codes - read more on the ICD9Data.com blog.