Hi, my name is Hanan Sela. I'm a third-year PM&R resident at Beaumont Hospital in Royal Oak, Michigan. My case is titled proximal muscle weakness, generalized rash, and idiopathic pulmonary fibrosis in an adult female with antisynthetase syndrome. So, just a little background, about a third of patients who have dermatomyositis or polymyositis actually have a group of clinical findings that are collectively known as antisynthetase syndrome. This is an autoimmune disease due to autoantibodies that target aminoacyl-tRNA synthetases. Some of these antibodies include anti-GL-1, PL-7, PL-12, amongst many others. These patients present with proximal muscle weakness, polyarthritis, interstitial lung disease, rash, and Raynaud's phenomenon. Now, jumping into the case here, I had a patient who was a 67-year-old female at the time. This was last year while I was on inpatient rehab. Now, she has a past medical history of pulmonary fibrosis, fibromyalgia, and arthritis and presented to the hospital with this diffused, pruritic rash on her chest, on her hands, on her legs. It was pretty symmetric, but most prominent in her hands. She had bilateral lower extremity edema and proximal muscle weakness as well. So, lab workup revealed an elevated ESR, CRP, lactic acid dehydrogenase, and creatine phosphokinase. Dermatology was consulted. They had obtained a biopsy of her skin that showed spongiosis and inflammation, but not convincing evidence of dermatomyositis. She was treated as having examinist dermatitis, and she actually had improvement with that. Now, rheumatology was also consulted, and this patient was started on IV steroids due to concern for dermatomyositis, and she actually had improvement in proximal muscle weakness. It was slow improvement, but nonetheless, she got a little better. She had an EMG done by our department, which revealed evidence of a myopathy in both the left upper and lower extremity, and she had a muscle biopsy obtained that eventually resulted with type 2 fiber atrophy, but it did not show inflammation or vasculitis to suggest dermatomyositis. So, she was obviously transitioned to inpatient rehab for intense therapy, where she had further improvement in her proximal muscle weakness, and during that time, a myositis panel that was initially ordered revealed a positive finding for anti-PL12 antibody, which is essentially diagnostic for antisensitivity syndrome. When rheumatology saw this result, they recommended that the patient follow up with them in their clinic after discharge to be started on a disease-modifying agent. Now, there's a lot of takeaways from this case. Some things to note is that, you know, patients who have antisensitivity syndrome, specifically those who have anti-JO1 or anti-PL12, have been observed to have antisensitivity syndrome associated with interstitial lung disease who actually lack evidence of myositis. Now, anti-PL12 and PL7 antibodies are characterized by more severe lung involvement. So, these are just very important kind of correlations to make, especially if you have a patient with one of these antibodies. They definitely have prognostic implications. Now, patients who have anti-aminoacyl tRNA synthetases, such as the ones I mentioned earlier in this case report, amongst many others, have been shown to have more frequent arthritis, fever, interstitial lung disease, and mechanic's hands. If the lungs are involved, the severity and the type of the lung injury determines the prognosis. Now, although a lot of these patients are initially started on steroids for acute therapy, their treatment often requires immunosuppressant medications and antisensitivity syndrome. This patient of ours was later discharged. She followed up with rheumatology and was actually started on mycophenolate molphthalate. So, the reason why this diagnosis is not only important to kind of keep in the back of your mind when you have a patient with proximal muscle weakness, but also it has prognostic implications, as I mentioned earlier. A lot of times they will have corticosteroid-resistant myositis. And in her case, interstitial lung disease requiring immunosuppressive therapy. So, the takeaway from all of this is if you have a patient that you have a high clinical suspicion for dematomyositis or polymyositis, or furthermore, are actually diagnosed, they should routinely undergo anti-aminoacyl-tRNA synthetase autoantibody search because it can certainly impact their prognosis. So, I hope you found this case interesting and thank you for listening. Hope you all enjoy the rest of the conference. Thank you.

proximal muscle weakness

generalized rash

antisynthetase syndrome

autoimmune disease

mycophenolate mofetil