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Rheumatology

Rheumatology

Clinical discussions on autoimmune diseases, biologic therapies, vasculitis, and musculoskeletal conditions.

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How do you treat MAS in patients with systemic JIA or AOSD with HLA-DRB1*15 alleles given risk for DRESS hypersensitivity to IL1 or IL6 inhibitor therapy?

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Rheumatology · University of Alabama Birmingham

Tough question. HLA-DRB1*15 is pretty common, and it may be a risk allele for lung disease. I, and many others, are not convinced, however, the lung disease represents DRESS, nor that a range of biologics are the etiology of the lung disease. One of my most recent sJIA patients presented with high e...

Would you consider combination belimumab and anakinra for a patient who has SLE and MAS?

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Rheumatology · Massachusetts General Hospital

I have used the combination of belimumab and IL-1 inhibition in a patient with SLE to treat recurrent pericarditis. It would not be unreasonable to use anakinra for MAS and add this to their belimumab. Managing MAS requires treatment of the underlying driver of the MAS, which in this case seems to b...

How do you utilize anifrolumab for SLE in your practice?

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Rheumatology · Uniformed Services University of the Health Sciences (USUHS)

The answer to this SLE question involves a complex decision-making process. It is such an important question! I apologize in advance for the long answer. I am in the camp of achieving remission in my SLE patients and getting them off prednisone in the safest ways possible (Fanouriakis et al., PMID 3...

How do you approach symptom control in a patient with diffuse cutaneous systemic sclerosis (dcSSc) and rapidly progressive skin disease?

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Rheumatology · Mayo Clinic

This patient clearly seems to be on the uphill trajectory for skin involvement in RNA Pol III+ dcSSc which can progress quite rapidly over 6-12 months. Pain, burning, tightness and pruritus are quite intense during this period, and can be very difficult to treat. It does improve somewhat with time, ...

How do you determine which patients with ANCA associated vasculitis may be good candidates for reduced dose glucocorticoid tapering?

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Rheumatology · Massachusetts General Hospital

This is an important question. Currently there are several groups of patients that benefit from reduced dose glucocorticoids: The largest group are patients who are receiving avacopan for remission induction. The ADVOCATE trial demonstrated that avacopan can markedly reduce the dose of glucocorticoi...

Would you continue belimumab in high risk lupus patients with overlapping scleroderma features with ILD who become pregnant and who are already on HCQ and azathioprine?

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Rheumatology · Uniformed Services University of the Health Sciences (USUHS)

If "high risk" means at high risk of flare or increased disease activity, then "yes." I always have a shared decision-making conversation with the patient first. This almost always occurs well before conception/pregnancy, as I strongly believe in contraception and pregnancy conversations early in th...

Do you recommend discontinuing IVIG for a newly diagnosed HMGCR+ statin induced necrotizing myopathy who developed a recent brachial DVT?

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Rheumatology · University of Pittsburgh

Really, the issue is if the DVT was provoked or not. If possibly not provoked then would decrease the dose of IVIG or give it over a long time frame. For example, if the patient is on 2 gm/kg over 2 days, I would do 2 gm/kg over 4 days. Or reduce the dose to 1 gm/kg over 2 days.

How would you approach management of a patient with ESRD on the transplant list who is found to have high titer APS labs (ACL, B2GP1, LAC)?

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Rheumatology · SUNY Upstate Medical University

This is an excellent but rather complex question. Management of patients with ESRD on the transplant list who are found to have high titer APS labs (ACL, B2GP1, LAC) depend on the renal histology, underlying autoimmune disease history, and comorbidities. Patients with thrombotic manifestations resul...

How do you approach weakly positive PL-7 antibody in a patient who initially presented with muscle weakness, rhabdomyolysis and non specific muscular edema on MRI that resolved with IV fluids?

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Rheumatology · The University of Texas Health Science Center at Houston (UTHealth)

During an episode of rhabdomyolysis, muscle MRI isn't reliable since it would be positive regardless of the underlying cause. So, monitoring the trajectory of CPK levels and serial muscle exams to evaluate for weakness would offer a more reliable assessment for true myositis. Positive antibody resul...

What dose and duration of steroid therapy do you employ while starting mycophenolate in an outpatient with relatively stable CTD-ILD?

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Pulmonology · Massachusetts General Hospital

This is a great question since it raises some important concepts about treatment response in this patient population. Providers often talk about the presence or absence of a steroid-responsive process, and if so, whether a steroid-sparing agent would be beneficial. My bias is to avoid steroids in an...