Secondary amyloidosis (type AA) is rarely encountered but can be a significant complication of ankylosing spondylitis (AS) and may lead to proteinuria and renal dysfunction. Anti-tumor necrosis factor-alpha (anti-TNFα) agents may be used to induce clinical remission by suppressing systemic inflammation in secondary amyloidosis. The patient described , with the diagnosis of AS, was diagnosed with secondary amyloidosis, despite treatment with disease modifying anti-rheumatic medication. He developed marked proteinuria, renal dysfunction and low levels of serum albumin. Diagnosis of amyloidosis was confirmed by renal biopsy. During a 2-year treatment period with etanercept, an anti-TNFα agent, a definite improvement was determined in all parameters. This case illustrates that in the treatment of secondary amyloidosis related to AS, etanercept, an anti-TNF α agent, can be considered an effective therapeutic option.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Deeper response predicts better outcomes in high-risk-smoldering-myeloma: results of the I-PRISM phase II clinical trial.
Nat Commun
January 2025
Center for Early Detection and Interception of Blood Cancers, Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, MA, USA.
Early therapeutic intervention in high-risk smoldering multiple myeloma (HR-SMM) has shown benefits, however, no studies have assessed whether biochemical progression or response depth predicts long-term outcomes. The single-arm I-PRISM phase II trial (NCT02916771) evaluated ixazomib, lenalidomide, and dexamethasone in 55 patients with HR-SMM. The primary endpoint, median progression-free survival (PFS), was not reached (NR) (95% CI: 57.
View Article and Find Full Text PDFNephrotic Syndrome as a Complication of Systemic Calcitonin Amyloidosis From Long-Standing Metastatic Medullary Thyroid Cancer.
AACE Clin Case Rep
August 2024
Section of Endocrinology, Diabetes, Nutrition & Weight Management, Boston University Chobanian & Avedisian School of Medicine, Boston, Massachusetts.
Background/objective: Medullary thyroid cancer often results in elevated calcitonin levels, which can cause localized formation of calcitonin amyloid, though rarely complications of systemic calcitonin amyloidosis have been reported. The objective of this report is to encourage awareness of calcitonin amyloid causing nephrotic syndrome in patients with metastatic medullary thyroid cancer.
Case Report: A 65-year-old woman with weakness, fatigue, anasarca, anemia, thrombocytopenia, venous and arterial thrombi, and a cavitary right lung lesion was transferred for care.
Waldenström Macroglobulinemia-Induced Cardiac Amyloid Light Chain Amyloidosis.
Ochsner J
January 2024
Division of Cardiology, Baylor Scott & White Medical Center, Temple, TX.
Waldenström macroglobulinemia is a rare cancer of plasma cells characterized by the excessive production of immunoglobulin M (IgM). IgM-associated systemic amyloid light chain (AL) amyloidosis is a rare complication of Waldenström macroglobulinemia, characterized by the misfolding of lambda light chains that deposit in various organs, including the heart. We describe a case of progressive nonischemic cardiomyopathy secondary to Waldenström macroglobulinemia and IgM-associated AL amyloidosis that was refractory to medical therapy and highlight the challenges in diagnosis and management.
View Article and Find Full Text PDFPrognostic value of left atrial strain in patients with stage II light-chain cardiac amyloidosis receiving cardiac magnetic resonance imaging: a prospective cohort study.
Quant Imaging Med Surg
December 2024
Department of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Background: The prognostic significance of left atrial (LA) dysfunction in cardiac amyloidosis is being increasingly acknowledged, but its importance in stage II light-chain cardiac amyloidosis (AL-CA) remains unclear. This study aimed to determine the prognostic value of LA strain in stage II AL-CA.
Methods: Patients with stage II AL-CA who underwent cardiac magnetic resonance at Peking Union Medical College Hospital between January 1, 2015, and October 31, 2021, were consecutively enrolled in this cohort study.
Dual transcatheter edge-to-edge repair in a patient with cardiac amyloidosis and severe secondary mitral and tricuspid regurgitation: a case report.
Eur Heart J Case Rep
December 2024
Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Hufelandstr. 55, 45147 Essen, Germany.
Background: Mitral and tricuspid regurgitation in patients with cardiac amyloidosis (CA) pose significant diagnostic and therapeutic challenges due to its non-specific symptoms and limited treatment options. Transcatheter edge-to-edge repair (TEER) is complicated by altered cardiac geometry, advanced restriction, and potential amyloid valve deposits.
Case Summary: We present the case of dual TEER in a 79-year-old male with advanced transthyretin cardiac amyloidosis (ATTR-CA) and severe symptomatic mitral and tricuspid regurgitation.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!