Diagnosis Of Systemic Amyloidosis Research Articles

AL–AMYLOIDOSIS RELATED ADVANCED HEART FAILURE DIAGNOSED THROUGH F–18–FLUTEMETANOL PET SCAN

Abstract Background Systemic amyloidosis (SA) is a group of rare diseases in which abnormal misfolded proteins, amyloid fibrils, accumulate in tissue. The gold standard for diagnosis is tissue biopsy of the affected organ, but it is invasive and needs specific proteomic or immunohistochemistry analyses to characterize the involved protein. We report a case of a complex diagnosis of SA in an advanced heart failure (HF) patient. Methods and Results A 68–year–old woman with history of recurrent uveitis started to complain dyspnea in 2022, after Covid19. Since her symptoms worsened, in June 2023 she was diagnosed in another center with restrictive cardiomyopathy with preserved left ventricular (LV) ejection fraction. She underwent coronary angiography (unremarkable), cardiac MRI (aspecific fibrosis), and a FDG–PET scan which showed non–homogeneous LV fixation. The patient was treated with steroids for the suspicion of an immunologic–inflammatory disease, without benefit. Fourteen months after the initial presentation, she was admitted to our centre for advanced inotrope–dependent HF. She had mildly increased LV hypertrophy and severe systolic dysfunction with restrictive physiology and, notably, peri–orbital subcutaneous hemorrhages (Figure 1). Hematological screening for amyloidosis showed elevated serum free lambda light chains. Repeated cardiac MRI scan was consistent with infiltrative disease. A total–body PET scan with dual tracer was performed. The control session with F–18–FDG showed mild aspecific cardiac uptake in the basal septum and lateral wall (Figure 2), while off–label use of F–18–Flutemetanol, an Alzheimer‘s disease approved experimental tracer that binds to amyloid fibrils beta sheets with very high sensitivity and specificity, showed intense cardiac, hepatic, pulmonary and muscular uptake, with wash–out–timing diagnostic for AL–SA (Figure 3). Periumbilical fat biopsy confirmed the clinical suspicion of SA. Hematological investigations led to the diagnosis of stage I–A lambda micromolecular multiple myeloma, with systemic amyloidosis and a IIIb cardiac prognostic score. Conclusions Endomyocardial biopsy is an invasive procedure and periumbilical fat biopsy discriminates the amyloid protein type only by proteomic analysis, which is available in very few Italian centres. The off–label use of Alzheimers’ PET tracers could reduce the delay of cardiac AL–SA diagnosis, allowing early disease identification and prompting timely hematological therapy.

S3086 New Diagnosis of Systemic Amyloidosis in a Patient With Chronic Hepatitis B and Elevated Liver Stiffness Score on MR Elastography

S3086 New Diagnosis of Systemic Amyloidosis in a Patient With Chronic Hepatitis B and Elevated Liver Stiffness Score on MR Elastography

Abstract WMP81: Association Between Systemic Amyloidosis And Intracranial Hemorrhage

Introduction: Isolated amyloid deposition in the brain is associated with intracranial hemorrhage. Whether systemic amyloidosis also increases the risk of intracranial hemorrhage is unclear. Methods: We evaluated the association between systemic amyloidosis and intracranial hemorrhage using claims data from a 5% national sample of Medicare beneficiaries from 2008-2015. The primary outcome was non-traumatic intracranial hemorrhage, defined as a composite of intracerebral hemorrhage, subarachnoid hemorrhage, and subdural hemorrhage. Secondary outcome were each hemorrhage type assessed separately. The exposure and outcomes were identified using previously validated ICD-9-CM diagnosis codes. We used Cox regression analysis adjusting for demographics and vascular risk factors to evaluate the association between systemic amyloidosis and intracranial hemorrhage. We also examined the risk of hip fracture (negative control). In sensitivity analyses, we excluded patients with cardiac amyloidosis, a subset most likely to be on antithrombotic therapy. Results: Among 1.8 million Medicare beneficiaries, 924 were diagnosed with systemic amyloidosis. During a median follow-up of 5.3 years (IQR, 2.8- 6.7), the cumulative incidence of intracranial hemorrhage was 19 per 1,000 patients per year among patients with amyloidosis, and 2 per 1,000 patients per year in those without amyloidosis. In adjusted Cox models, systemic amyloidosis was associated with an increased risk of intracranial hemorrhage (HR, 4.3; 95% CI, 2.9-6.3). The association persisted in a sensitivity analysis after excluding beneficiaries with cardiac amyloidosis (HR, 8.0; 95% CI, 5.0-12.7). In secondary analyses, systemic amyloidosis was associated with intracerebral hemorrhage (HR, 5.6; 95% CI, 3.6-8.7), subarachnoid hemorrhage (HR, 14.7; 95% 9.0-24.0), and subdural hemorrhage (HR, 3.6; 95% 2.0-6.2). There was no association between systemic amyloidosis and hip fracture (HR, 0.9; 95% CI, 0.6-1.4). Conclusions: In a large, heterogeneous national cohort of elderly patients, a diagnosis of systemic amyloidosis was associated with a 4-fold increased risk of intracranial hemorrhage, including intracerebral, subarachnoid, and subdural hemorrhages.

Carcinoma de lábio mimetizando queratoacantoma: relato de casos

ABSTRACT Oral amyloidosis is a disease characterized by extracellular and irreversible deposition of amorphous and fibrillar proteins in the oral cavity, being strongly associated with Multiple Myeloma. The objective of this study is to report a case of a 62-year-old woman diagnosed with Multiple Myeloma who, approximately 2 years after starting treatment for the underlying disease, presented a lesion on the lateral border of the tongue with exophytic growth, pinkish color, vascularized, painless, measuring 3cm in its largest diameter. After histopathological analysis through incisional biopsy, a final diagnosis of amyloidosis was obtained. As a local treatment, we opted for complete excision of the lesion. The patient evolved to death due to Multiple Myeloma influenced by the diagnosis of systemic amyloidosis. Oral amyloidosis is usually associated with the systemic presentation of the disease, making it necessary to conduct a thorough investigation of other organs. Its diagnosis is important since the prognosis is directly related and can negatively influence survival rates and treatment of the underlying disease.

Amiloidose lingual associada a mieloma múltiplo: relato de caso

ABSTRACT Oral amyloidosis is a disease characterized by extracellular and irreversible deposition of amorphous and fibrillar proteins in the oral cavity, being strongly associated with Multiple Myeloma. The objective of this study is to report a case of a 62-year-old woman diagnosed with Multiple Myeloma who, approximately 2 years after starting treatment for the underlying disease, presented a lesion on the lateral border of the tongue with exophytic growth, pinkish color, vascularized, painless, measuring 3cm in its largest diameter. After histopathological analysis through incisional biopsy, a final diagnosis of amyloidosis was obtained. As a local treatment, we opted for complete excision of the lesion. The patient evolved to death due to Multiple Myeloma influenced by the diagnosis of systemic amyloidosis. Oral amyloidosis is usually associated with the systemic presentation of the disease, making it necessary to conduct a thorough investigation of other organs. Its diagnosis is important since the prognosis is directly related and can negatively influence survival rates and treatment of the underlying disease.

S2260 The Role of Endoscopic Examination and Biopsy in the Diagnosis of Systemic Amyloidosis: A Case Report

S2260 The Role of Endoscopic Examination and Biopsy in the Diagnosis of Systemic Amyloidosis: A Case Report

Utility of abdominal skin punch biopsy for detecting systemic amyloidosis.

Early and accurate diagnosis of systemic amyloidosis (SA) is critical for optimal patient outcomes. Biopsy of clinically uninvolved skin and subcutaneous tissue including abdominal skin punch biopsy (ASPB) is often used as a surrogate for affected organ sampling. There is a lack of published data on the sensitivity and specificity of ASPB for diagnosing SA. Retrospective chart review between 2000 and 2020 of all ASPB was performed to diagnose SA. Amyloid deposition was confirmed by Congo red stain. Study group includes patients with histopathologically and clinically confirmed diagnosis of SA. Control group includes patients without histopathology of amyloid deposition and no clinical SA. Forty-one patients meeting inclusion criteria were analyzed; 23 study group and 18 control group patients. The overall diagnostic sensitivity of ASPB was 43% (95% CI 23%-66%) and the specificity 100% (95% CI 81%-100%). The AL amyloidosis diagnostic sensitivity was 64% (95% CI 35%-87%). ASPB >10 mm in depth had 100% (95% CI 54%-100%) sensitivity compared to 24% for depth ≤10 mm (P=.002). ASPB is a minimally invasive and highly specific method of diagnosing SA. It is particularly sensitive for diagnosing AL amyloidosis and the diagnostic sensitivity can be significantly improved with adequate biopsy depth and diameter.

Detection of cardiac amyloidosis by PET/CT imaging using 124I-p5+14 peptide

Abstract Introduction Systemic amyloidosis is characterized by the deposition of protein fibrils in abdominothoracic organs, notably the heart, leading to organ dysfunction and significant morbidity. Patients who present with light chain (AL) amyloid-associated cardiomyopathy have a poor prognosis and median survival of only ∼ 9 mos. Cardiac amyloidosis is also present in many of the other forms of the disease and may be ever present in patients with transthyretin-associated amyloidosis (ATTR). Currently, no radiotracers are approved for the quantitative imaging of cardiac amyloid load. To address these needs, we have developed a synthetic amyloid-reactive peptide radiotracer, 124I-p5+14, suitable for PET/CT imaging. The peptide binds the three major forms of amyloid (AL, ATTR and ALECT2), as well as other, less common, types through multivalent electrostatic interactions with amyloid-associated glycosaminoglycans and fibrils. Herein we report safety, dosimetry, and efficacy data on the first 22 patients from the ongoing Phase 1, first-in-human trial of 124I-p5+14 in patients with systemic amyloidosis (NCT 03678259). Methods Patients >18 years of age with a confirmed diagnosis of systemic amyloidosis and not requiring heparin therapy are eligible. Subjects received <2 mg of 124I-p5+14 (<2 mCi) administered as a single IV bolus. PET/CT images for the initial cohort (n=3) were acquired from 25 min to 48h post injection. The second cohort of patients were imaged at ∼5 h and 24 h post injection. Image data were acquired using a Biograph 16 PET/CT scanner with a low dose CT. Uptake of radiotracer in the left ventricular wall was performed by automated image segmentation and standard uptake value ratios (SUVR) were calculated using blood pool as the reference tissue. Results To date, 22 patients (13 AL, 5 ATTR, and 4 other) patients have been evaluated. The gender-averaged mean whole body effective dose was 0.24 mSv/MBq. Cardiac uptake of the radiotracer was visually detected by a reader blinded to the patients' organ involvement in 85% and 100% of patients with AL and ATTR respectively, including patients with asymptomatic cardiac involvement - no cardiac symptoms or elevated cardiac biomarkers. The mean myocardium SUVR for visually positive AL and ATTR patients were 2.2±0.6 and 2.6±0.4. For visually negative AL patients the SUVRs were 1.0 and 0.9. In addition to cardiac amyloid, 124I-p5+14 uptake was observed in the nerves, ligaments, liver, spleen, adrenal glands, kidneys, pancreas, pituitary, and lung, with overall abdominothoracic organ-specific sensitivity of >90% based on clinical presentation. Sensitivity in the heart was 100%. Conclusions PET/CT imaging of 124I-p5+14 provides excellent visualization of AL and ATTR cardiac amyloidosis which can be readily quantified as a means of monitoring response to therapy or disease progression. The 124I-p5+14 radiotracer was also capable of detecting amyloid in other abdominothoracic organs. AL and ATTR cardiac amyloidosis Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): National Heart Lung and Blood Institute, National Institutes of Health; ACTP Gift Fund at the UTGSM

Amyloid associated infiltrative cardiomyopathy with Waldenstrom’s Macroglobulinemia

Amyloid associated infiltrative cardiomyopathy with Waldenstrom’s Macroglobulinemia - IJPO- Print ISSN No: - 2394-6784 Online ISSN No:- 2394-6792 Article DOI No:- 10.18231/j.ijpo.2019.135, Indian Journal of Pathology and Oncology-Indian J Pathol Oncol

Incidental multiple adenopathies and the diagnosis of systemic amyloidosis

This report describes the clinical case of a 67-yearold man with hypertension referenced to the medical consultation for the study of several bilateral mediastinal adenopathies discovered accidentally on a chest computed tomography (CT) following the patient’s admission to the emergency department after falling off a tree. Of note in the physical examination is the presence of macroglossia and thick lips (Figure 1A). In the complementary study of adenopathies serologic data and a positron emission tomography (PET) scan were requested. Analytically with a sedimentation rate of 21 mm, the peripheral blood smear analysis was normal and there was a good renal function with no change in phosphocalcic metabolism. At the level of immunoglobulins, a value of IGG-2300 mg/dl was found with the respective gamma-monoclonal peak (IGG-lambda) in the electrophoresis allowing the diagnosis of monoclonal gammopathy of undetermined significance. Posteriorly, PET scan demonstrated additional cervical and mediastinum-hilar bilateral ganglia with low metabolic activity of inflammatory nature, without suggestive alterations of tumor lesions with significant hypermetabolism of 18F-FDG (Figure 1B, Figure 1C). The definitive diagnosis of primary amyloidosis was obtained later through abdominal fat biopsy (Figure 1D). An echocardiogram was requested to rule out the cardiac involvement of the disease. Given the clinical stability of the patient and the absence of significant findings in the complementary diagnostic tests, the strategy adopted in this patient was “watch and see.”

Nail Biopsy in the Diagnosis of Systemic Amyloidosis

A 60-year-old woman with a 13-year history of monoclonal gammopathy of unknown significance (MGUS) presented with worsening nail dystrophy. Prior workup for systemic amyloidosis had been unrevealing, and no other signs of cutaneous disease were present. Nail biopsy was consistent with amyloid deposition, and the patient subsequently underwent autologous hematopoietic stem cell transplantation for AL amyloidosis. In light of the growing literature regarding nail changes as a presenting sign of systemic amyloidosis and the promising utility of nail biopsy, we suggest a low threshold for biopsy in appropriate patients when nail changes characteristic of amyloidosis are refractory to conventional treatment.

Characteristics of South Korean Patients with Hereditary Transthyretin Amyloidosis.

Background and PurposeThis retrospective cross-sectional study included 18 patients from unrelated families harboring mutations of the transthyretin gene (TTR), and analyzed their characteristics and geographical distribution in South Korea.MethodsThe included patients had a diagnosis of systemic amyloidosis, clinical symptoms, such as amyloid neuropathy or cardiomyopathy, and confirmation of a TTR gene mutation using genetic analysis recorded between April 1995 and November 2014.ResultsThe mean age at disease onset was 49.6 years, and the mean disease duration from symptom onset to diagnosis was 3.67 years. Fifteen of the 18 patients were classified as mixed phenotype, 2 as the neurological phenotype, and only 1 patient as the cardiac phenotype. The most-common mutation pattern in South Korea was Asp38Ala, which was detected in eight patients. Thirteen patients reported their family hometowns, and five of the eight harboring the Asp38Ala mutation were from the Gyeongsang province in southeast Korea. The other eight patients exhibited a widespread geographical distribution. A particularly noteworthy finding was that the valine at position 30 (Val30Met) mutation, which was previously reported as the most-common TTR mutation worldwide and also the most common in the Japanese population, was not detected in the present South Korean patients.ConclusionsSouth Korean patients with hereditary TTR amyloidosis exhibited heterogeneous TTR genotypes and clinical phenotypes. The findings of this study suggest that the distribution of TTR amyloidosis in South Korea is due to de novo mutations and/or related to the other countries in East Asia.

Patterns of Treatment Failure in Systemic Light Chain Amyloidosis with Long-Term Survival after Chemotherapy

BackgroundThe prognosis of systemic light chain (AL) amyloidosis is poor, which is associated early death assiated with cardiac involvement. It was reported that patients who avoided earth death have better survival than myeloma patients. However, the patterns of treatment failure of AL amyloidosis patients with long-term survival have not been evaluated. The aim of this study was to analyze the clinical outcome of patients with AL amyloidosis who survived more than 1 year and experienced treatment failure after systemic chemotherapy.MethodsIn this retrospective cohort study, 165 patients with AL amyloidosis received chemotherapy including ASCT in Samsung medical center between September 1997 and September 2015 were analyzed. The conditioning chemotherapy of ASCT was high-dose melphalan (200mg/m2). The patients who survived more than 1 year and showed disease progression or death were included in the study population. Progression was defined as first hematologic or organ progression after diagnosis of systemic amyloidosis. Hematologic or organ progression was evaluated according to the Roundtable on Clinical Research in Immunoglobulin Light-chain Amyloidosis. The progression-free survival and overall survival were analyzed.ResultsA total of 112 patients who survived more than 1 year during the study period with median follow-up 50.3 months, 7 patients was lost to follow-up and 47 patients did not showed progression. 58 patients experienced death or progression and they were eligible for analysis. 14 of 58 (24.1%) patients received ASCT, 13 (22.4%) patients received bortezomib-based regimens, 6 (10.3%) patients received oral melphalan with dexamethasone before progression. 4 (6.9%) patients achieved hematologic complete remission (CR) and 5 (8.6%) patients achieved hematologic very good partial response (VGPR), all of 9 patients demonstrated cardiac progresion. Otherwise, 27 of 47 patients (57.4%) without progression achieved hematologic CR or VGPR. Among 54 patients who did not achieve hematologic CR, 24 (44.4 %) patients showed hematologic progression and 28 (51.9%) patients showed cardiac progression, kidney progression was 17 (31.5%) and liver progression was 3 (5.6%), respectively. There were 4 patients with hematologic progression followed by cardiac progression, and the median time interval from hematologic progression to cardiac progression was 2.6 months. 39 deaths (67.2%) were identified in cohort and most common cause of death was cardiac progression (12 of 39 deaths - heart failure, fatal arrhythmia, cardiac arrest).ConclusionMore than half of patients in this cohort received ASCT or bortezomib based chemotherapy, cardiac progression was a leading cause of treatment failure in systemic AL amyloidosis with long-term survival (>1 year), even in the patients with hematologic CR. Continuation of chemotherapy or ASCT beyond hematologic response might be needed to achieve cardiac response and long-term survival in patients with AL amyloidosis who demonstrated cardiac progression. Further studies to elucidate the efficacy of chemotherapy beyond hematologic response are warranted. DisclosuresNo relevant conflicts of interest to declare.

Systemic Amyloidosis with Predominant Spine Involvement: A Case Report

Purpose: Primary systemic amyloidosis with main bone involvement is a rare disease. Clinical symptoms and radiographic findings are usually nonspecific and may be confused with primary bone tumor, metastatic disease, metabolic disorders or infections. The occurrence of an amyloidoma in the spine is rare. Methods: We describe the case of a 57-year old man presenting with a pathological fracture of T7 in presence of a large, soft tissue mass narrowing the spinal canal, responsible for a worsening paraplegia. Diagnosis of systemic amyloidosis was made after surgical treatment. Pathological examination showed an amorphous eosinophilic material, positive staining with Congo red, birefringence under polarized light relating to amyloid, with the presence of rare plasma cells. Results: After surgical procedure patient’s symptoms improved but with incomplete neurological recovery. PET-CT scan revealed multiple bone locations without, at the beginning, extra-skeletal involvement. The patient underwent oncological and surgical treatment with progression of the disease and visceral involvement. He died two years after the diagnosis. Conclusions: Bone involvement during systemic amyloidosis is rare and often underestimated, it had predominantly visceral involvement (kidney, heart, liver, gastrointestinal tract, lung) and unfavorable clinical course if not treated. Appropriate histopathologic studies are an essential step to define diagnosis and treatment of these patients. Treatment consists of chemotherapy, steroids, autologous blood stem cell transplantation and biologic antiinflammatory drugs. Spine localization can bring to nerves compression or pathological fracture and, in these cases, surgical treatment has a role to improve patient’s quality of life.

Systemic Amyloidosis in a Patient with Type 2 Diabetes Mellitus as a Uncommon Cause of Non-Diabetic Renal Disease

Background: The incidence of non-diabetic renal disease is very high in type 2 diabetic patients. Systemic amyloidosis as one of the non-diabetic renal disease rarely occur in type 2 diabetes mellitus subjects and may have the high risk of early mortality. Case report and management: We report a case in a 66-year-old patient with legs edema, inappetence and frothy urine for 2 weeks. The patient was diagnosed type 2 diabetes on routine testing 10 years ago without any symptoms. The blood glucose was adequately controlled with diet and exercise. Physical examination showed edema of lower extremities and hepatomegaly. 24 hrs urinary protein was 4.7 g. Blood investigation showed slightly impaired liver function while other results incluing serological studies and bone marrow aspirate showed negative or in normal range. Funduscopy showed normal retinal blood vessels and optic disc. Echocardiography revealed cardiac amyloidosis with septal hypertrophy and the ultrasound scan revealed hepatomegaly. Hepatic biopsy was performed and diagnosis of systemic amyloidosis was made due to positive congo red staining. Conclusions: When massive proteinuria occurred in patient with type 2 diabetes and other internal organs like liver and heart are affected, superimposed systemic amyloidosis should be considered in particular

Patient with primary systemic amyloidosis presenting with intractable diarrhoea

Introduction: Primary systemic amyloidosis is the most common form of systemic amyloidosis. Clinical presentation commonly involves organs such as the kidney and heart. We report on a patient with systemic amyloidosis presenting as intractable diarrhoea. Numerous investigations had to be done before the diagnosis could be made highlighting the challenge in making a diagnosis due to the slow progression of the disease. This report is to increase awareness among physicians of this diagnosis and to emphasise the importance of identifying patients quickly. Case Presentation: A 63 year old male was admitted with a six months history of intractable, watery diarrhoea, anorexia and progressive renal failure. He had been investigated for proteinuria and deranged renal function prior to admission but no therapy was suggested and the diagnosis at that time was inconclusive. Extensive laboratory and radiological investigations were done. The diagnosis of systemic amyloidosis was made on serum free light chains which showed excess lambda chains and a terminal iliac and colonic biopsy which revealed eosinophilic thickening of the blood vessels with positive Congo red stain. Unfortunately the patient died prior to definitive management for the amyloidosis. Conclusion:

18F-FDG-PET-CT with Little Value in Different Diagnosis between Pulmonary Malignancy and Amyloidosis

Pulmonary amyloidosis is rare and is often misdiagnosed due a lack of general awareness of the condition. The role of PET-CT in the differential diagnosis between pulmonary amyloidosis and lung malignant tumors is still unclear. Herein, we describe a 61-year-old Chinese woman who presented with a right lower lobe pulmonary nodule, and right hilar and mediastinal lymph nodes shadows. On PET-CT examination, the patient was found to have right lower lung malignant lesions with multiple right hilar and mediastinal lymph node metastases. She was diagnosed with nephrotic syndrome with a history of pathologic type of amyloidosis over the previous two months. After performing right thoracotomy, a histopathologic diagnosis of pulmonary amyloidosis was made. The patient has done well postoperatively, showing no local recurrence in the lung or deterioration of her condition in a 12- month follow-up period. The pathology of nephrotic syndrome in patients with amyloidosis led to the diagnosis of systemic amyloidosis being made in this patient. From this case, combined with the domestic and international literature, we conclude that PET-CT is of little value for the different diagnosis between pulmonary amyloidosis and lung malignant tumors.

Localized insulin‐derived amyloidosis: A potential pitfall in the diagnosis of systemic amyloidosis by fat aspirate

Localized insulin‐derived amyloidosis: A potential pitfall in the diagnosis of systemic amyloidosis by fat aspirate

Diagnosis of systemic amyloidosis and amyloidosis mediated cardiomyopathy by VATS pleural biopsy for chronic pleural effusion.

Amyloidosis is a family of diseases characterized by the extracellular accumulation of amyloid protein, causing altered physiology based on its abnormal deposition in an organ. The etiology of persistent pleural effusions in patients with systemic amyloidosis is unknown. Endomyocardial biopsy is the gold standard of diagnosis for patients with cardiac involvement in systemic amyloidosis. We present the case of a patient with systemic amyloidosis whose diagnosis was made by pleural pathology collected via video-assisted thoracic surgery after a false negative endomyocardial biopsy.

Skeletal scintigraphy indicates disease severity of cardiac involvement in patients with senile systemic amyloidosis

BackgroundSenile systemic amyloidosis (SSA) is a common aging phenomenon in the elderly population. Nevertheless, pre-mortem diagnosis of SSA is rare. Thus, data on clinical characterization and disease severity are limited. Methods36 consecutive SSA patients (71.6 [64.7–82.7]years) were evaluated by electrocardiography, echocardiography, laboratory tests, and 99mTechnetium-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) scintigraphy (n=20). ResultsIn addition to cardiac involvement, amyloid deposition was found in rectum (n=6), peripheral nerves (n=2), and urinary bladder (n=2). Five patients showed low voltage pattern. Thickness of interventricular septum (IVS) was 20 [12–27]mm. LV longitudinal function was diminished (TDI-s 5 [3–11] cm/s; MAPSE 6.5 [2.5–19]mm; TAPSE 12.5 [2–24]mm). LV systolic function (LV-EF<45%) was markedly decreased in 19 patients. Plasma levels of troponin T (0.05 [0.01–0.23]µg/L) and NT-proBNP (4318 [205–16597]ng/L) were elevated. 99mTc-DPD heart retention was 7.8 [2.4–11.0]% and correlated with MAPSE (ρ=−0.716; p=0.0018), TAPSE (ρ=−0.491; p<0.05), and IVS (ρ=0.556; p=0.0153). Heart-to-body ratio correlated with MAPSE (ρ=−0.771; p=0.0018), IVS (ρ=0.603; p=0.0086).Twelve patients died during follow-up of 27.4 [0.1–106.2]months. Exclusively 99mTc-DPD heart retention, diastolic dysfunction and in trend MAPSE were associated with patient's outcome. Interestingly, risk predictors that were well established in patients with AL amyloidosis were not predictive for survival in patients with SSA. ConclusionsThis study gave first evidence that 99mTc-DPD HR may be capable to display the extent of cardiac amyloid deposition. Moreover, this study suggested that 99mTc-DPD HR, diastolic dysfunction and in trend MAPSE are associated with poor outcome. Nevertheless, these findings need to be established in a larger prospective trial.