Amyloidosis Secondary

Cystic fibrosis, AA (secondary) amyloidosis Amyloid A protein Systemic... 

In one series of some 5500 patients with juvenile rheumatoid arthritis, 105 were found to have developed secondary amyloidosis 37 of the latter had been receiving sodium aurothiomalate. In 12 of these children the time between withdrawal of gold (because of adverse effects) and the finding of amyloid A was less than six months (SEDA-21,237). 

Clinically, amyloidosis is classified (omitting some rare forms) into five main groups (1) primary amyloidosis, (2) amyloidosis associated with multiple myeloma, (3) secondary amyloidosis associated with inflammatory or infectious diseases, (4) a form associated with aging, and (5) familial amyloidosis. Increased knowledge of the chemical structure of the deposits has led to a revised classification that takes into consideration the type of amyloid protein present. Tins classification is used in Table 20-14. 

Iq21-q23 1.05 APCS Amyloid P component, serum 104770 Secondary amyloidosis... 

Amyloid was not found in fhe skin biopsies of several of our patients with heroin related secondary amyloidosis, although interestingly, multiple pulmonary nodules due to AA amyloid have been described in an HIV positive intravenous drug abuser [31a]. 

As with heroin-induced focal segmental glomerulosclerosis, the interstitial cell inflammation is more prominent in the biopsies of patients with substance abuse amyloidosis than in other forms of secondary amyloidosis [22]. Accompanying the tubulointerstitial involvement are a number of physiologic and clinical... 

The clinician can determine whether a patient such as Katta Bolic is j mounting an acute phase response to some insult, however subtle, by deter- mining whether several unique acute phase proteins are being secreted by the liver. C-reactive protein, so named because of its ability to interact with the C-polysaccharide of pneumococci, and serum amyloid A protein, a precursor of the amyloid fibril found in secondary amyloidosis, are elevated in patients undergoing the acute phase response and as compared with healthy individuals. Other proteins normally found in the blood of healthy individuals are present in increased concentrations in patients undergoing an acute phase response. These include haptoglobin, certain protease inhibitors, complement components, ceruloplasmin, and fibrinogen. The elevated concentration of these proteins in the blood increases the erythrocyte sedimentation rate (ESR), another laboratory measure of the presence of an acute phase response. 

Urinary tract A 72-year-old woman with rheumatoid arthritis, secondary amyloidosis, and steroid-induced diabetes mellitus, who was taking glucocorticoids and low-dose methotrexate, developed an emphysematous pyelonephritis [76 ]. 

Fig. 4.19. The complete amino acid sequence of an amyloid protein extracted from tissues of a patient with secondary amyloidosis associated with familial Mediterranean fever. The sequence is not related to that of an immunoglobulin. There is some uncertainty at position 53, which may be Arg. From Levin el al. (134).

Levin et al. were unable to find any amyloid of nonimmunoglobulin nature in the deposits of three patients with multiple myeloma or mac-roglobulinemia. However, the converse was not true, since some material related to L chain was found in patients with secondary amyloidosis, whose deposits were mainly nonimmunoglobulin in nature. This immunoglobulin-like material appeared heterogeneous, in contrast to that seen in myeloma patients. Relatively few individuals with secondary amyloidosis have been examined at this writing for the presence of amyloid related to immunoglobulin. 

Tuglular S, Yalcinkaya F, Paydas S, et al. A retrospective analysis for aetiology and clinical findings of 287 secondary amyloidosis cases in Turkey. Nephrol Dial Transplant 2002 17(11) 2003-2005. 

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