COVID-19 aggravates limb ischemia in Systemic Scleroderma patients: A case report

Juvenile Scleroderma is one of the rare autoimmune diseases of the connective tissue. The concurrence of this autoimmune disorder with COVID-19 can cause limb ischemia because both disease entities are pro-inflammatory and pro-thrombotic. There needs to be more data in this context, and there needs to be more case reports describing the symptomatology and disease course in juvenile Scleroderma and COVID-19 patients.

Addressing and investigating the above concern, a case study was presented by a team of researchers led by Mark Jason D. C. Milan and colleagues from the College of Medicine and Philippine General Hospital.

The case was presented as follows:

• An adolescent with acute limb ischemia presented and had generalized hypo-and-hyperpigmented skin lesions history.
• The patient also had a mild, non-productive cough.
• She tested positive for SARS-CoV-2 on a nasopharyngeal swab detected by RT-PCR.
• The tests revealed elevated anti-phospholipid antibodies, anti-nuclear antibodies, and D-dimer.
• Protein S activity was low.
• There was evidence of peripheral arterial disease in imaging studies.
• The patient started peripheral vasodilators, Methotrexate, and anticoagulation.
• The researchers monitored affected limbs and other organs closely.
• After regular infusion of Cyclophosphamide, limb ischemia was controlled after four months.
• The multi-disciplinary care was continued for the patient.

To conclude, there is the interplay of COVID-19 hyperinflammatory state, and rheumatologic disorders and this knowledge in this context is evolving.

COVID-19 exacerbates cutaneous manifestations of autoimmune disorders via antigen protein mimicry and cytokine imbalance. COVID-19 and systemic sclerosis exhibit elevated D-dimer, which owe to their pro-thrombotic sequela.

More data must be collected on the association of Protein S activity with COVID-19 and systemic sclerosis. However, more studies are warranted to arrive at a consensus on thrombosis prophylaxis for Scleroderma and COVID-19 patients.

Further reading:

Milan, MJDCMJDC, Dans, LFLF & Torres-Ticzon, V.M.F. Acute limb ischemia in an adolescent with COVID-19 and systemic scleroderma: a case report. B.M.C. Pediatr 22, 737 (2022). https://doi.org/10.1186/s12887-022-03761-w