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And the 3rd morning of #BSR21 is kicking off. I'm starting the day with "Cardiac complications of rheumatic Disease and anti-rheumatic therapies". The first talk is by @TYoungstein on "Infiltrative cardiomyopathy in the rheumatology clinic" 
Much focus on accelerated atherosclerosis in rheumatic patients but less coverage has been focused on cardiac inflammation and Infiltrative diseases - this needs to change!
Be alert to cardiac involvement in new patients and those with established disease
#BSR21 @TYoungstein
Be alert to cardiac involvement in new patients and those with established disease#BSR21 @TYoungstein
Myocardial inflammation:
*Underdiagnosed
*Delays in diagnosis
*Requires tx escalation
*Check cardiac biomarkers, ECG, echo, CMR, nuclear medicine imaging and endomyocardial biopsies as required
#BSR21 @TYoungstein
*Underdiagnosed
*Delays in diagnosis
*Requires tx escalation
*Check cardiac biomarkers, ECG, echo, CMR, nuclear medicine imaging and endomyocardial biopsies as required
#BSR21 @TYoungstein
She highlights that the report of echos & CMRs in the assessment of myocardial inflammation and infiltration are a specialist area and important to liaise with your local departments as to whether they are doing the right assessments & have the right expertise
#BSR21 @TYoungstein
#BSR21 @TYoungstein
Infiltrative cardiomyopathies:
*Can be secondary to inherited or acquired conditions
*Adverse prognosis is in part due to delays in diagnosis
*Check troponin & BNP
*Be alert to the possibility in those with abnormalities such as diastolic dysfunction on echo
#BSR21 @TYoungstein
*Can be secondary to inherited or acquired conditions
*Adverse prognosis is in part due to delays in diagnosis
*Check troponin & BNP
*Be alert to the possibility in those with abnormalities such as diastolic dysfunction on echo
#BSR21 @TYoungstein
4 key infiltrative cardiomyopathies
*Iron overload (haemochromatosis)
*Sarcoidosis
*Amyloid
*Fabrys
#BSR21 @TYoungstein
*Iron overload (haemochromatosis)
*Sarcoidosis
*Amyloid
*Fabrys
#BSR21 @TYoungstein
Sarcoid infiltrative cardiomyopathy:
*Cardiac involvement in 25-30%
*Can cause CHB & ventricular arrhythmias
*It is treatment responsive (immunosuppression) & can benefit from supportive care like pacemakers
*Patchy involvement (be aware of this if biopsying)
#BSR21 @TYoungstein
*Cardiac involvement in 25-30%
*Can cause CHB & ventricular arrhythmias
*It is treatment responsive (immunosuppression) & can benefit from supportive care like pacemakers
*Patchy involvement (be aware of this if biopsying)
#BSR21 @TYoungstein
Fabrys disease and Infiltrative cardiomyopathy:
*Lysosomal storage disease
*Men and women affected (but x-linked)
*Arthralgia and arthritis
*Rash
*Renal failure
*30% of adult patients initially diagnosed with rheumatic disease
https://ard.bmj.com/content/79/6/e71
#BSR21 @TYoungstein
*Lysosomal storage disease
*Men and women affected (but x-linked)
*Arthralgia and arthritis
*Rash
*Renal failure
*30% of adult patients initially diagnosed with rheumatic disease
https://ard.bmj.com/content/79/6/e71
#BSR21 @TYoungstein
Amyloid infiltrative cardiomyopathy:
*AA: known rheumatic disease (though they present with proteinuric renal failure & cardiac involvement only happens very late on)
*AL: myalgia, purpura, neuropathy, raised ESR; plasma cell dyscrasia
*Wild type transthyretin
#BSR21 @TYoungstein
*AA: known rheumatic disease (though they present with proteinuric renal failure & cardiac involvement only happens very late on)
*AL: myalgia, purpura, neuropathy, raised ESR; plasma cell dyscrasia
*Wild type transthyretin
#BSR21 @TYoungstein
Wild type transthyretin amyloid:
*Mean age at onset 74
*88%male
*Typical heart failure symptoms; normal EF
*Not a rare disease
*Bilateral carpal tunnel syndrome heralds symptom onset 12 years before cardiac symptoms
*Other features: spinal stenosis, OA
#BSR21 @TYoungstein
*Mean age at onset 74
*88%male
*Typical heart failure symptoms; normal EF
*Not a rare disease
*Bilateral carpal tunnel syndrome heralds symptom onset 12 years before cardiac symptoms
*Other features: spinal stenosis, OA
#BSR21 @TYoungstein
Cardiac amyloidosis diagnosis and treatment guidelines here:
https://academic.oup.com/eurheartj/article/42/16/1554/6212698
#BSR2 @TYoungstein
https://academic.oup.com/eurheartj/article/42/16/1554/6212698
#BSR2 @TYoungstein
And here this excellent infographic from the above paper on how and when one might suspect cardiac amyloidosis and the subsequent steps to take:
#BSR21 @TYoungstein
#BSR21 @TYoungstein
