Case highlights challenges of treating SSc after COVID-19

Similar symptoms and lab results complicate process of diagnosis/treatment

Patricia Inácio, PhD avatar

by Patricia Inácio, PhD |

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A woman developed scleroderma, also known as systemic sclerosis (SSc), after she contracted COVID-19, according to a case report that highlights the challenges of treating complex autoimmune conditions.

Treatment with a corticosteroid led to scleroderma renal crisis, “highlighting the risk of steroid use in all patients with systemic sclerosis,” scientists wrote.

The shared symptoms and blood work results between SSc and post-COVID-19 syndrome pose a challenge that can lead to “delayed diagnosis and treatment, resulting in increased healthcare burden, and increased morbidity and mortality for patients,” researchers noted.

The report, “Systemic sclerosis following COVID-19 infection with recurrent corticosteroid-induced scleroderma renal crisis,” was published in the journal BMJ Case Reports.

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Autoimmune disorders on the rise after COVID-19

Evidence is increasing about the rise of autoimmune disorders following infection by SARS-CoV-2, the virus that causes COVID-19. Although the mechanisms are not fully understood, the viral infection is believed to affect the immune system and antibody response. However, evidence for increased risk for SSc after COVID-19 is scarce.

“There are currently only two reported cases of systemic sclerosis occurring post-COVID-19 infection,” the researchers wrote.

In the study, researchers in Australia describe a patient who developed SSc after she got COVID-19. The woman, in her 40s, had newly developed Raynaud’s phenomenon, a condition in which the fingers and toes feel numb, prickly, and frigid in response to cold temperatures or stress.

In addition, she experienced pain in the joints of her hands and tightening of her fingers. These symptoms developed two weeks after she recovered from a mild case of COVID-19.

The doctor suspected post-viral reactive arthritis and autoimmune response. Lab work revealed she had anti-nuclear antibodies, which are found in autoimmune disorders. These autoantibodies are directed against proteins in the cell’s nucleus.

The patient was treated with the corticosteroid prednisolone at a dose of 25 mg daily and was referred for outpatient rheumatology follow-up. After three weeks, she went to an emergency department and reported progressive fatigue, dyspnea (shortness of breath), headache, and abdominal pain.

Her body mass index (a measure of body fat) was 31, indicative of obesity, but she had no other disorders or family history of autoimmune diseases.

Examinations revealed signs of sclerodactyly — a type of skin tightening common in SSc — that extended above the wrist. Skin hardening was also found on her trunk and face. Additionally, she had telangiectasias, which are dilated blood vessels near the skin surface.

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Tests reveal signs of acute kidney damage

Blood work revealed signs of acute kidney damage. A CT scan of the abdomen and pelvis showed no abnormalities. The patient received into-the-vein fluids and continued the prednisolone regimen.

However, within 48 hours of admission, her condition worsened: she developed severe high blood pressure, anemia (low hemoglobin, the protein that carries oxygen in red blood cells), and low platelet counts.

The presence of schistocytes, or fragmented red blood cells, confirmed a diagnosis of microangiopathic hemolytic anemia, a process of red blood cell destruction.

While clinicians considered potential diagnoses, the patient developed tonic-clonic seizures — which involve both stiffening and twitching phases of muscle activity. She was then intubated and transferred to the intensive care unit.

She received treatment to control her blood pressure, as well as plasma exchange, a procedure that filters the blood to remove harmful autoantibodies, until doctors could rule out thrombotic thrombocytopenic purpura, which is a serious blood disorder wherein blood clots form in small blood vessels throughout the body.

Since corticosteroid use has been linked to scleroderma renal crisis, treatment with prednisolone was discontinued.

The patient’s blood pressure lowered and her red blood cell destruction eased. CT scans of the chest showed fluid buildup, but no signs of scarring. An echocardiogram revealed high pressure in the pulmonary arteries.

She tested positive for anti-topoisomerase I, an SSc-related autoantibody. Overall, the clinical findings led to a diagnosis of diffuse SSc.

Next, the patient was placed on intermittent hemodialysis (a treatment to clean the blood) and underwent a kidney biopsy, which confirmed kidney damage and poor blood flow.

Over the next two months, her condition improved and she was weaned from the hemodialysis. A few weeks later, her breathing difficulties worsened, and new fluid was accumulating around her heart, which was considered a cardiac manifestation of SSc.

She was re-started on corticosteroid therapy, which resolved the cardiac problem but triggered scleroderma renal crisis.

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Woman also diagnosed with pulmonary hypertension

Treatment was administered to resolve the patient’s acute kidney failure, but further tests confirmed a diagnosis of pulmonary hypertension, a condition characterized by narrowing of the pulmonary arteries, restricting blood and oxygen flow, and rising blood pressure. She received Revatio (sildenafil), a medication that opens (dilates) blood vessels, with good response.

However, she still had reduced urine volume and had to undergo dialysis. Treatment for high blood pressure was also maintained.

The patient was ineligible for a kidney transplant due to progression of her pulmonary hypertension and intolerance to corticosteroids. She continued to experience nausea and abdominal discomfort. Further examination revealed impaired function of the esophagus, likely a result of SSc. The esophagus is the tube that connects the throat with the stomach.

Due to recurrent aspiration events — when food or liquids enter the airways by accident — treatment with immunosuppressants was delayed. She was also off disease-modifying therapies.

“Given the complexity of the case with multiple systems affected, the patient will be followed up by several specialty units including nephrology, rheumatology, respiratory and gastroenterology,” the researchers wrote, adding that “future management decisions will require a multidisciplinary approach.”