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It& #39;s Wednesday! It& #39;s answer time! The correct answer was Cyclophosphamide. Congratulations to 45% of you who were correct! Now let& #39;s think about what this questions is about... #RespEd #eosinophilia 3/n
As @LewisStanding1 suggested the diagnosis here is EGPA. Severe asthma is a differential but does not explain all the symptoms/signs. There is multiorgan involvement so it& #39;s essential to recognise & treat quickly 4/n
Eosinophilic Granulomatosis with PolyAngiitis was previously called Churg-Strauss syndrome. This is now outdated terminology & should be avoided. Eponymous names are unhelpful for clinicians, students & patients. 5/n
The rename was part of the 2012 Chapel Hill revised nomenclature for vasculitides: https://onlinelibrary.wiley.com/doi/pdf/10.1002/art.37715.">https://onlinelibrary.wiley.com/doi/pdf/1... EGPA is an ANCA-associated small vessel vasculitis 6/n
Neither Churg nor Strauss have any known Nazi associations, that was Wegener who was a member of the Sturmabteilung. Wegener wasn’t even the first person to describe GPA, misattribution & bias being yet more reasons why eponyms more broadly must go https://www.healio.com/news/rheumatology/20190418/whats-in-a-name-how-wegeners-granulomatosis-became-gpa.">https://www.healio.com/news/rheu... 7/n
Anyway, back to EGPA. The 1990 @ACRheum diagnostic criteria remain clinically useful. They are under review as we learn more: https://www.sciencedirect.com/science/article/pii/S0953620515001442">https://www.sciencedirect.com/science/a... #RespEd #vasculitis #eosinophlia 8/n
Our case fulfils several of these criteria - poorly controlled #asthma, #eosinophilia, vasculitic neuropathy suggested by sensation loss on foot, infiltrates on CT, facial pain suggesting sinus disease. The raised Trop & abnormal CMR show cardiac involvement 9/n
But wait, she& #39;s ANCA negative, we hear you cry! Yes, EGPA is ANCA-associated but only 30-70% patients are ANCA positive. https://www.vasculitis.org.uk/about-vasculitis/what-is-anca">https://www.vasculitis.org.uk/about-vas... #RespEd #RheumEd #vasculitis 10/n
Eosinophilic lung diseases are fascinating. Think #asthma #EGPA, #ABPA #parasitic infections, acute & chronic eosinophilic pneumonia, drug reactions & more. Imaging provides important clues - see @Radiopaedia https://radiopaedia.org/articles/eosinophilic-lung-disease-1?lang=gb">https://radiopaedia.org/articles/... 11/n
And for a deep dive into EGPA #pathophysiology, #epidemiology, #immunology, #genetics, & #histopathology this is a great review https://www.frontiersin.org/articles/10.3389/fimmu.2014.00549/full">https://www.frontiersin.org/articles/... #RespEd 12/n
OK onto treatment! Our patient would be given steroids (Pred or IVMP) then they need steroid-sparing immunosuppression due to the multiorgan involvement incl cardiac which otherwise has a poorer prognosis. There are a lack of high quality RCTs - scope for more #research 13/n
Here& #39;s a treatment recommendation from the EGPA consensus taskforce. Life or organ threatening disease requires rapid control, & cyclophosphamide is a good option - https://www.sciencedirect.com/science/article/pii/S0953620515001442">https://www.sciencedirect.com/science/a... 14/n
The 5 factor score can be used to predict #prognosis. Adjunctive cytotoxic drugs are recommended to treat FFS ≥ 1 high-risk EGPA patients (although RCTs lacking). https://journals.lww.com/md-journal/Fulltext/2011/01000/The_Five_Factor_Score_Revisited__Assessment_of.2.aspx">https://journals.lww.com/md-journa... #RespEd #vasculitis 15/n
Azathioprine or methotrexate would be options for longterm remission maintenance of EGPA after induction with steroids & cyclophosphamide. Rituximab can be considered for ANCA positive patients with relapse or refractory disease but is not 1st line #RespEd 16/n
What about Benraluzimab, a treatment for severe #asthma. Biologics such as Benra & Mepoluzimab have been used to treat EGPA & been shown to be effective in small case series https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7152676/">https://www.ncbi.nlm.nih.gov/pmc/artic... but not NICE approved & not 1st line. 17/n
EGPA diagnosis & treatment continues to evolve, & we recommend you consult an experienced severe #asthma specialist & a #Rheumatologist with an interest in vasculitis for all EGPA patient treatment decisions. #phoneafriend #RespEd #team #vasculitis #eosinophilia 18/n
Ok that& #39;s it for this week& #39;s #MCQMonday. It has reaffirmed the #eosinophil as the best white cell (don& #39;t @me @teamneutrophil) Pls share our handle with any #RespEd enthusiasts ( #phlegmfriends). See you next monday for more! #breathe #loveyourlungs #FOAMed #SCE #RespisBest 19/19
