Extracellular Volume Associates With Outcomes More Strongly Than Native or Post-Contrast Myocardial T1.

OBJECTIVES: Because risk stratification data represents a key domain of biomarker validation, we compared associations between outcomes and various cardiovascular magnetic resonance (CMR) metrics quantifying myocardial fibrosis (MF) in noninfarcted myocardium: extracellular volume fraction (ECV), native T1, post-contrast T1, and partition coefficient. BACKGROUND: MF associates with vulnerability to adverse events (e.g., mortality and hospitalization for heart failure [HHF]), but investigators still debate its optimal measurement; most histological validation data show strongest ECV correlations with MF. METHODS: We enrolled 1,714 consecutive patients without amyloidosis or hypertrophic cardiomyopathy from a single CMR referral center serving an integrated healthcare network. We measured T1 (MOdified Look-Locker Inversion recovery [MOLLI]) in nonenhanced myocardium, averaged from 2 short-axis slices (basal and mid) before and 15 to 20 min after a gadolinium contrast bolus. We compared chi-square test values from CMR MF measures in univariable and multivariable Cox regression models. We assessed "dose-response" relationships in Kaplan-Meier curves using log-rank statistics for quartile strata. We also computed net reclassification improvement (NRI) and integrated discrimination improvement (IDI for Cox models with ECV vs. native T1). RESULTS: Over a median of 5.6 years, 374 events occurred after CMR (162 HHF events and 279 deaths, 67 with both). ECV yielded the best separation of Kaplan-Meier curves and the highest log-rank statistics. In univariable and multivariable models, ECV associated most strongly with outcomes, demonstrating the highest chi-square test values. Native T1 or post-contrast T1 did not associate with outcomes in the multivariable model. ECV provided added prognostic value to models with native T1, for example, in multivariable models IDI = 0.0037 (95% confidence interval [CI]: 0.0009 to 0.0071), p = 0.02; NRI = 0.151 (95% CI: 0.022 to 0.292), p = 0.04. CONCLUSIONS: Analogous to histological previously published validation data, ECV myocardial fibrosis measures exhibited more robust associations with outcomes than other surrogate CMR MF measures. Superior risk stratification by ECV supports claims that ECV optimally measures MF in noninfarcted myocardium.

Can videolaryngoscopy be a first option in a patient with laryngeal amyloidosis? / ¿Puede la videolaringoscopia ser una primera opción en paciente con amiloidosis laríngea?

Amyloidosis is a term that involves a group of diseases characterised by deposition of extracellular monoclonal light-chain fibrillar immunoglobulin aggregates in the body, including many organs, with the larynx among them. A case is presented of a 78 year-old man who was referred to our institution for strangulated umbilical hernia treatment. He suffered from progressive hoarseness and dysphagia for 5months. He had a history of primary laryngeal amyloidosis. Awake intubation was performed successful with the King Vision® video-laryngoscopy. Sedation was achieved using a remifentanil infusion and midazolam. Haemorrhagic lesions are caused by deposition of amyloid in and around vessels, resulting in increased vascular fragility. Therefore, anaesthetists should take care in intubating the tracheas of these patients.

Serum amyloid P component scintigraphy and turnover studies for diagnosis and quantitative monitoring of AA amyloidosis in juvenile rheumatoid arthritis.

OBJECTIVE: To evaluate aspects of the natural history of AA amyloidosis complicating juvenile rheumatoid arthritis (JRA), and its response to therapy with chlorambucil. METHODS: Scintigraphy and 7-day turnover studies were performed in JRA patients with histologically proven (n = 35) or clinically suspected (n = 30) AA amyloidosis, following intravenous injection of 123I and 125I-labeled serum amyloid P component (SAP). Prospective monitoring studies were performed over 2-3 years in 20 patients with amyloidosis. All but 2 amyloidosis patients were treated with chlorambucil. RESULTS: Positive scanning results were obtained in all patients in whom imaging was performed within 12 years of positive biopsy findings of amyloid and in 5 patients with clinically suspected amyloidosis. Negative scanning results with normal SAP metabolism, indicating regression of amyloid, were obtained in 4 patients whose amyloidosis had been in full clinical remission for more than 12 years. Prospective monitoring studies in patients whose JRA-associated inflammatory activity was in remission demonstrated regression of amyloid in 8 patients and no substantial changes in 8 others; however, in 4 further patients with active inflammation, there was accumulation of amyloid. There was a very poor correlation between the amount of amyloid present at a particular site and the resultant organ dysfunction. CONCLUSION: Radiolabeled SAP scintigraphy and turnover studies are useful complementary tools in the diagnosis, screening, and quantitative monitoring of type AA amyloidosis in JRA. The amyloid deposits may progress and/or regress at different rates in different anatomic sites over short periods.

[Rheumatological complications of dialysis]. / Les complications rhumatologiques des dialysés.

The articular complications observed in dialysed chronic renal failure failures, whose incidence increases with the duration of dialysis, are closely correlated with the development of beta 2-microglobulin amyloidosis, responsible for nerve tunnel syndromes, arthralgia and chronic joint swelling with frequently multiple subchondral cysts on x-rays. Microcrystalline pathology is dominated by apatite deposits, which may also be involved in the pathogenesis of destructive arthropathy. Articular complications with destruction of the large joints or involvement of the first carpometacarpal joint interfere with the functional prognosis. Sepsis must be excluded in cases of destructive cervical spondyloarthropathies. The pathogenesis of destructive arthropathies is probably multifactorial, consisting of apatite and amyloid deposits, secondary hyperparathyroidism and aluminium poisoning.

Double contrast small bowel enema in a case of selective duodeno-jejunal amyloidosis.

Double contrast small bowel enema in a patient with selective amyloidosis of the duodenum and jejunum revealed an unusual radiologic pattern, mimicking multiple polyposis. Many submucosal polypoid lesions, sessile or pedunculated, were scattered throughout the duodenum and proximal jejunum. The lesions had smooth borders and did not show any retraction at the base. Other typical radiologic patterns of intestinal amyloidosis described in the literature were not observed in the present case. Differential diagnosis from multiple polyposis and diffuse lymphoproliferative neoplastic diseases is discussed.

Bone scan in systemic amyloidosis.

Thirty patients diagnosed as having systemic amyloidosis (six with primary amyloidosis and 24 with secondary amyloidosis) were given bone scans with 99Tcm-methylene diphosphonate in order to evaluate the skeletal and extra-osseous uptake and their clinical-scintigraphic correlation. Extra-osseous uptake appeared in nine instances, more frequently in primary (5/6) than in secondary (4/24) amyloidosis. Fourteen patients had clinical and echocardiographic suspicion of cardiac amyloidosis but only one showed cardiac uptake. Six patients had probable hepatic involvement but in only two cases was hepatic uptake seen. The extra-osseous uptake suggests the presence of amyloid deposits in the organs and soft tissues which take up the bone tracer. The absence of any uptake does not rule out amyloid infiltration.

Significance of abnormal gastrointestinal gas in systemic amyloidosis.

Nine cases of amyloidosis involving the gastrointestinal tract were reviewed for the presence of abnormal gastrointestinal gas by taking plain roentgenograms of the abdomen (PRA) as a diagnostic adjunct. Findings were compared to data obtained by conventional barium study or endoscopic examination. PRA revealed marked gaseous dilatation of the gastrointestinal tract simulating intestinal obstruction in three cases and a moderate or mild increase of gas in two cases each. Abnormality was thus found in seven of the nine cases by PRA. Conventional barium study by means of upper gastrointestinal series, small bowel series and barium enema showed abnormal findings in five of seven cases. Endoscopic examination of the stomach and distal colon disclosed mucosal abnormality in three of seven cases. The rate of abnormal findings detected by the three methods cannot easily be compared to each other because of the differences in symptoms and treatment of individual patients. Nevertheless, PRA seem to be valuable in demonstrating a high rate of abnormalities in amyloidosis.

Amyloidosis masquerading as inflammatory bowel disease with a mass lesion simulating a malignancy.

Our patient, who was known to have multiple myeloma, presented with weight loss, rectal bleeding, and a barium enema study suggestive of a colitis with a mass lesion. Colonoscopy with biopsy revealed the mass to be large mucosal folds infiltrated with amyloidosis. Amyloidosis has been reported to mimick malignancy, mainly by tumorous deposits in the stomach and less commonly in the small and large bowels. Gastrointestinal surgery in patients with amyloidosis potentially may have undesirable consequences due to failure of anastomotic suture lines and subsequent sepsis (6, 11, 17, 18). The knowledge that amyloidosis may be associated with multiple myeloma and an appreciation of the wide range of gastrointestinal roentgenographic findings in patients with amyloidosis should prompt the clinician to obtain endoscopic and biopsy evaluation of these patients.