COVID-19 linked to reduced blood flow in girl with scleroderma
Case study reports on patient's successful treatment with vasodilators
COVID-19 prompted blood vessel damage and a reduced blood flow to the limbs — a condition known as ischemia — in a girl with scleroderma (SSc), a case report described.
The girl was ultimately discharged from the hospital after four weeks of inpatient treatment with blood vessel wideners known as vasodilators, according to researchers.
“We present this case to demonstrate that COVID-19 can aggravate limb ischemia in patients with systemic scleroderma,” the team wrote, adding that their report “adds to the evolving knowledge of the interaction between COVID-19 and hyperinflammation in a rheumatologic disorder.”
The report, “Acute limb ischemia in an adolescent with COVID-19 and systemic scleroderma: a case report,” was published in the journal BMC Pediatrics.
COVID-19 seen to prompt blood vessel damage, reduced blood flow
Acute limb ischemia is characterized by a rapid reduction in blood supply that threatens limb viability. It can occur in a severe form in people with COVID-19 and scleroderma, as both of these conditions promote inflammation and clotting.
However, no study has reported on the symptoms and disease course of COVID-19 in patients with juvenile scleroderma.
Here, researchers at the University of the Philippines described the case of an adolescent girl with scleroderma who was infected with SARS-CoV-2, the virus that causes COVID-19, and developed acute limb ischemia.
The girl had first been treated, 20 months before her hospitalization, for generalized red skin patches. These patches had developed into areas of reduced or increased pigmentation — commonly referred as a salt-and-pepper pattern. She often experienced joint stiffness, had weight loss, and had bluish fingers when exposed to cold temperatures.
The patient also had persistent areas of dry skin and was seen in an outpatient clinic for suspicion of systemic scleroderma, but was then lost to follow-up.
In the interval prior to her admission, the girl was diagnosed with Raynaud’s phenomenon (RP), a condition in which the fingers and toes feel numb, prickly, and frigid in response to cold temperatures or stress. This condition progressed, the week before she was hospitalized, into reduced blood flow in the right hand, and bluish fingers and toes.
She went back to the rheumatology clinic, and was prescribed Revatio (sildenafil) and Norvasc (amlodipine), a calcium channel blocker used to lower blood pressure.
However, ischemia continued to progress. She was brought to the emergency department and despite being awake and calm, she was unable to walk due to tenderness in her toes.
Clinical examination was uneventful, but her skin continued showing color changes, and she had sensory deficits in her hands and feet. In fact, an exam confirmed a 50% sensory deficit across several phalanges – the little bones that make the bony core of fingers and toes. All toes in the right foot were affected.
At this point, she tested positive for COVID-19. She was hospitalized to manage limb ischemia, with a goal toward preventing amputation.
The girl was treated with the calcium channel blocker nifedipine, given every six hours, along with Revatio three times a day. A nitroglycerin patch was used on the affected fingers and toes.
The researchers noted that iloprost — commonly used to widen blood vessels and improve blood circulation — was the first option, but was unavailable at the hospital.
She also started treatment with the anti-inflammatory prednisone and naproxen sodium as a pain reliever, but both were eventually discontinued.
The co-occurrence of COVID-19 and scleroderma were “postulated to have triggered the limb ischemia,” the researchers wrote.
We present this case to demonstrate that COVID-19 can aggravate limb ischemia [reduced blood flow] in patients with systemic scleroderma.
The girl began treatment with enoxaparin, a medication for blood clotting, administered under the skin (subcutaneously) every 12 hours. She experienced worsening sensory deficits in two fingers of the left hand, but not progression of ischemia. Further exams suggested arterial disease of the arms and legs.
After her 14-day quarantine due to COVID-19, she was transferred to the non-COVID ward for continued management. She received methotrexate, twice a day, for a week, and folic acid for her diffuse systemic scleroderma. For the persistent skin involvement, she was prescribed cyclophosphamide along with uromitexan, a medication used to prevent and reduce the toxicity of cyclophosphamide.
The girl was seen by the orthopedics team that recommended surgery, but the parents refused. She continued methotrexate without adverse reactions.
An imaging test, called angiography, that allows visualization of blood vessels revealed a complete blockage in a main artery of the right foot, among other alterations. She kept treatment with the vasodilators, or blood vessel wideners, and improvements in blood flow at the extremities were seen after four weeks, or about one month.
No progression of ischemia was then seen, and she was discharged with regular follow-up for cyclophosphamide infusion on outpatient basis.
The researchers concluded that further studies are needed “to demonstrate associations objectively” and to “ultimately arrive at a consensus on thrombosis prophylaxis [blood clot prevention] for patients with scleroderma and COVID-19.”