Researchers looked at a patient with rheumatoid arthritis who developed transthyretin amyloid cardiomyopathy (ATTR_CM)-mediated heart failure, according to a case report recently published in Cureus.
There is evidence suggesting that chronic systemic inflammation can contribute to protein misfolding and amyloid aggregation.
What is ATTR-CM?
Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is a rare progressive disease of the heart muscle that leads to congestive heart failure. It occurs when the transthyretin protein produced by the liver is unstable. Symptoms include fatigue; shortness of breath; irregular heart rate or palpitations; swelling of the legs, ankles and stomach; brain fog; wheezing; and dizziness. It often goes underdiagnosed because of a lack of awareness and knowledge of the disease. There is currently no cure for ATTR-CM.
“This case highlights the clinical presentation, diagnostic process, and management of cardiac amyloidosis while exploring a potential link between RA and amyloid deposition,” the report’s authors said.
Read more about ATTR-CM testing and diagnosis
Currently, there are scarce studies regarding the association between rheumatologic conditions and ATTR-CM.
“The potential association between RA and ATTR amyloidosis underscores the need for further investigation into the role of chronic inflammation in amyloid deposition,” the authors said. “Early recognition and appropriate management of cardiac amyloidosis can improve patient outcomes and quality of life, particularly with the advent of disease-modifying treatments for ATTR.”
The case involved a 68-year-old patient with a rheumatoid arthritis diagnosis under control with leflunomide and etanercept. He sought medical assistance for progressive swelling in both legs and shortness of breath.
An initial physical exam revealed fluid accumulation (edema) in both legs and in the abdominal cavity (ascites). Furthermore, auscultation revealed bilateral crackles in the bases of both lungs, suggestive of pulmonary edema. Laboratory workup was relevant for elevated cardiac biomarkers such as N-terminal pro-brain natriuretic peptide (NT-proBNP) and troponin I, suggesting myocardial damage.
Some studies suggest that drugs such as leflunomide and etanercept could have detrimental cardiovascular effects.
But electrocardiographic findings upon admission were suggestive of an infiltrative cardiomyopathy, and subsequent transthoracic echocardiography confirmed the findings. The physicians observed significant ventricular wall findings that were more suggestive of amyloid pathology than hypertensive cardiomyopathy.
To confirm the ATTR-CM diagnosis, the physicians performed a technetium-99m pyrophosphate (PYP) scan, which revealed a heart-to-contralateral lung (H/CL) ratio of 1.37 and a semiquantitative visual score of 2 to 3. These findings were sufficient to confirm the diagnosis without a need for a biopsy.
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