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Diabetes Mellitus, Obesity, Lipoprotein Disorders and other Metabolic Diseases
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
Studies in several different forms of amyloidosis have shown that therapy that succeeds in reducing the supply of the amyloid fibril precursor protein helps to preserve organ function and can prolong survival substantially. For AL amyloidosis, this is treatment of the antibody producing plasma cell disease such as myeloma. For AA amyloidosis, it is treatment of the underlying inflammatory disorder. Supportive therapy for failing organ function can include dialysis and sometimes organ transplantation.
Rheumatology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Clarissa Pilkington, Kiran Nistala, Helen Lachman, Paul Brogan
Colchicine is the prophylactic treatment of FMF (licensed in USA >4 years old): Continuous use reduces/prevents symptoms of FMF in at least 95%.Almost completely eliminates the risk of developing AA amyloidosis.Mechanism is poorly understood; children respond to 0.25–2 mg/d.
The Noncollagenous Proteins of the Intervertebral Disc *
Published in Peter Ghosh, The Biology of the Intervertebral Disc, 2019
The prognosis of reactive AA amyloidosis is more favorable than for idiopathic or myeloma associated amyloidosis. The reported survival rates are about 2 years236 for the former and 4 months for the latter.237 Amyloidosis has been estimated to be the cause of 43 to 47% of deaths among European patients with JRA,238 while the lower incidence of this condition in the U.S. reflects the lower death rate there from JRA.215 The proportion of deaths caused by amyloidosis in adult onset rheumatoid arthritis is variable, but generally much lower than for JRA. Koota et al.239 reported that amyloidosis was the cause of deaths in 8% of rheumatoid arthritis patients examined at autopsy.
Multiple nodular pulmonary amyloidosis in a patient with rheumatoid arthritis
Published in Modern Rheumatology Case Reports, 2019
Michiko Morishita, Tomoko Kawabata, Keiji Ohashi, Yoshia Miyawaki, Haruki Watanabe, Ken-Ei Sada, Jun Wada
Amyloidosis is a rare disorder in which fibrillary proteins called “amyloids” are deposited in the extracellular spaces of organs and tissue [1]. Systemic amyloid A (AA) amyloidosis occurs due to the tissue deposition of serum AA (SAA). AA amyloidosis is known to occur in chronic inflammatory conditions of connective tissue diseases, such as rheumatoid arthritis (RA). The prevalence in systemic amyloidosis patients with RA ranges from 7% to 26% [2,3]. AA amyloidosis most commonly occurs in the kidneys and the gastrointestinal tracts and rarely occurs in the lungs [4]. A previous report found that pulmonary amyloidosis is much more difficult to diagnose by transbronchial lung biopsy (TBLB) than by open lung biopsy [5]. However, there are few reports on pulmonary amyloidosis diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), which is a minimally invasive method with a high diagnostic yield [6]. We herein report a case of RA of multiple nodular pulmonary amyloidosis that was diagnosed by EBUS-TBNA.
Nationwide survey of 199 patients with reactive amyloid a amyloidosis in Japan
Published in Amyloid, 2019
Yasuaki Okuda, Toshiyuki Yamada, Mitsuharu Ueda, Yukio Ando
A total of 199 patients with AAA were included in the present study. The underlying diseases of AAA were rheumatoid arthritis (60.3%), uncharacterized inflammatory disorders (11.1%), neoplasms (7.0%), other rheumatic diseases (6.5%), inflammatory bowel diseases (4.5%), chronic infection (4.5%), Castleman’s disease (4.0%), and autoinflammatory diseases (2.0%). The clinical manifestations at the diagnosis of AAA were moderate to severe renal dysfunction (46.2%), moderate to severe proteinuria (30.7%), intractable diarrhea (32.2%), melena (4.5%), paralytic ileus (3.5%), heart failure (11.6%), cardiac conduction disturbances (10.1%), arrhythmia (5.5%), and hypothyroidism (11.6%). Diagnostic biopsies were performed most frequently in the gastrointestinal tract (66.3%), followed by the kidneys (22.1%), heart (5.5%), abdominal fat (4.0%), and others (3.0%). Biologics were used to treat 97 patients with AA amyloidosis (48.7%). Tocilizumab (TCZ) was administered to 66 patients, with 95.5% showing good responses. Anti-TNF agents were administered to 27 patients, with 74.1% showing good responses. The treatment effects of TCZ were significantly superior to those of anti-TNF agents (p < .007).
AA amyloidosis – Benefits and prospects of IL-6 inhibitors
Published in Modern Rheumatology, 2019
Among the complications of chronic inflammatory diseases, amyloid A (AA) amyloidosis is one of the most severe because of its poor prognosis. AA amyloidosis commonly affects the kidneys and gastrointestinal tract, and is characterized by various clinical symptoms, such as progressive proteinuria as well as renal dysfunction and failure. Control of the underlying disease, i.e. the suppression of serum amyloid A (SAA) levels, is the most critical step in the treatment of AA amyloidosis. Immunosuppressants, such as methotrexate, azathioprine, cyclophosphamide, and moderate doses of prednisolone, have previously been used to accomplish this. However, the satisfactory suppression of SAA levels cannot be achieved in some active cases, and the functions of the affected organs deteriorate. The prognosis of patients in the advanced stages of AA amyloidosis is generally poor. The major causes of death are renal failure and infection. Some retrospective studies and case reports demonstrated that anti-tumor necrosis factor (TNF) therapies were useful against AA amyloidosis [1–5]. Although treatments with anti-TNF agents reduce acute-phase reactants, such as C-reactive protein (CRP) and SAA, in chronic inflammatory diseases, the complete normalization of these acute-phase proteins is low [5].