This is a follow up exam. She was examined in 2019 when she was very symptomatic and again recently after being treated with NSAIDS and disease-modifying antirheumatic drugs (DMARDs). Pediatric Rheumatologist didn’t tell me the exact drugs used for her.
The sacroiliitis is still active. The knee is much better.
What would you have read the knee out as (what diagnosis or differential)?
I’ll post tomorrow what entity she was diagnosed as and been treated for.
Pelvis. I left the dates on:
[11yofpel20192.jpg]
[11yofpel20191.jpg]
[11yonowpel4.jpg]
Knee:
[11yoF4.jpg]
[11yoF5.jpg]
[11yoF1.jpg]
[11yoF2.jpg]
Now only 2 images with residual pathology.
[11yofnow2.jpg]
[11yofnow3.jpg]
[11yofnow1.jpg]
[11yofnow4.jpg]
Phillip Tirman
Correct diagnosis that this patient has according to the pediatric rheumatologist: Chronic Recurrent Multifocal Osteomyelitis (CRMO) for the knee as an overlap syndrome with Juvenile Spondyloarthritis.
My partner Mauricio (hope you’re not embarrassed) read it as inflammatory, most likely CRMO, r/o infection.
Funny but the Rheumatologist launched into how terrible the name is and I told her I was about to say the same thing.
Correct diagnosis: Drs Clarke, Breidahl, Lambert, Crnkovich, Dwek, Cantarelli, and Aguiar.
I wanted to include useful comments by Dr. Rob Lambert. Following the comments I am including a couple of references (Thanks Dr. Crnkovich for the AJR article).
Hi Phil,
Very interesting case.
Several things:
1) Knee – Multifocal metaphyseal lesions with surrounding bone marrow inflammation ++, and soft ST inflammation + – much improved after Rx. I think there is a good chance this is CRMO (SAPHO spectrum) but Juvenile Chronic Arthritis is possible – however the disease is bone predominant, and much less synovitis / enthesopathy.
2) The SI joints may be normal – there are several imaging issues here:
a) Many centers do MRI of the SI joints treating them the same as peripheral joints and for water sensitive sequences use echo times that are PD or IW weighted as part of a ‘pelvis/hip/SIJ’ protocol. This is a huge problem for the SIJ. The SIJ are really part of the spine and no-one ever does PDFS in the spine, it is always T2FS – for good reasons including the red marrow predominance that appears bright on PD, and to some degree also on IW.
b) Even on properly T2W sequences (TE >80ms for SE and >55ms for STIR), ‘flaring’ of the SIJ bone marrow is a common normal variant and the physiology behind that is controversial. Flaring lasts much longer (older) in boys than girls. And peds hyaline cartilage can be very bright on PDFS.
So, I don’t know the MRI parameters for the pelvis MRIs, but what I can say is that the muscle signal is FAR too bright (i.e. not T2 weighted enough) and these SI joints could be normal – but I’d need to see the whole scan and play with the windowing.
I’d love to see the entire DICOM data sets for both pelvis MRI. If you have access to the DICOM data, I have a website that allows folk to upload anonymized scans for external review. The software does not require downloading and works inside the browser, and all the patient identifying info is removed at the senders end before transmission so there is no risk of releasing confidential patient info.
In Edmonton, Jacob Jaremko and I are both passionately interested in arthropathy research, especially the spondyloarthropathies (Jacob did both peds and MSK fellowships and is head of peds MSK and I’m head of adult MSK) and so I have attached a recent article re SIJ bone marrow flaring in children.
I’ll be interested to hear more about this one.
Cheers,
Rob
Editorial comment (my opinion and not necessarily those of anyone else except the pediatric rheumatologist that I spoke with): Perhaps the worst disease name in all of medicine. It doesn’t have to be chronic, nor recurrent, nor multifocal, and it is not infection but rather inflammation.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5006369/
https://www.ajronline.org/doi/pdf/10.2214/AJR.09.7212