A woman developed scleroderma, also known as systemic sclerosis (SSc), after contracting COVID-19, according to a case report that highlights challenges in treating complex autoimmune diseases.
Treatment with a corticosteroid resulted in scleroderma-kidney crisis, “highlighting the risk of steroid use in all patients with systemic sclerosis,” researchers wrote.
The shared symptoms and blood test 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,” the researchers found.
The report, “Systemic sclerosis after COVID-19 infection with recurrent corticosteroid-induced scleroderma renal crisis‘ was published in the magazine BMJ case reports.
Autoimmune diseases are on the rise after COVID-19
There is growing evidence of the rise in autoimmune diseases following infection with SARS-CoV-2, the virus that causes COVID-19. Although the mechanisms are not fully understood, it is believed that viral infection affects the immune system and antibody response. However, there is little evidence of an increased risk of SSc after COVID-19.
“Currently, there are only two reported cases of systemic sclerosis occurring after COVID-19 infection,” the researchers wrote.
In the study, researchers in Australia describe a patient who developed SSc after contracting COVID-19. The woman, in her 40s, had recently developed Raynaud’s phenomenon, a condition in which fingers and toes feel numb, tingly and icy in response to cold or stress.
She also experienced pain in the joints of her hands and tightening of her fingers. Those symptoms developed two weeks after she recovered from a mild case of COVID-19.
The doctor suspected post-viral reactive arthritis and an autoimmune reaction. Lab work revealed that she had antinuclear antibodies found in autoimmune diseases. These autoantibodies are directed against proteins in the cell nucleus.
The patient was treated with the corticosteroid prednisolone at a dose of 25 mg daily and referred for outpatient rheumatological follow-up. After three weeks, she went to an emergency room and reported progressive fatigue, dyspnea (shortness of breath), headache, and abdominal pain.
Her body mass index (a measure of body fat) was 31, indicating obesity, but she had no other disorders or family history of autoimmune diseases.
Research revealed signs of sclerodactyly – a type of skin tightening common in SSc – extending beyond the wrist. Hard skin was also found on her torso and face. She also had telangiectasias, dilated blood vessels near the surface of the skin.
Tests show signs of acute kidney injury
Blood tests showed signs of acute kidney damage. A CT scan of the abdomen and pelvis showed no abnormalities. The patient received intravenous fluids and continued prednisolone treatment.
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 the diagnosis of microangiopathic hemolytic anemia, a process of red blood cell destruction.
As clinicians pondered possible 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 was given 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, a serious blood disorder that causes small blood vessels to build up all over the place Blood clots form the body.
Because corticosteroid use has been associated with scleroderma-renal crisis, prednisolone treatment was discontinued.
The patient’s blood pressure dropped and the destruction of her red blood cells subsided. CT scans of the chest showed fluid buildup but no evidence of scarring. An echocardiogram showed high pressure in the pulmonary arteries.
She tested positive for anti-topoisomerase I, an SSc-related autoantibody. Overall, the clinical findings led to the diagnosis of diffuse SSc.
Next, the patient underwent intermittent hemodialysis (a treatment to cleanse 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 off hemodialysis. A few weeks later, her breathing difficulties worsened and new fluid pooled around her heart, which was thought to be a cardiac manifestation of SSc.
She was restarted on corticosteroid therapy, which resolved the heart problem but set off a scleroderma-kidney crisis.
Woman also diagnosed with pulmonary hypertension
Treatment was given to correct the patient’s acute kidney failure, but further testing confirmed the diagnosis of pulmonary hypertension, a condition characterized by narrowing of the pulmonary arteries, restriction of blood and oxygen flow, and rising blood pressure. She was given Revatio (sildenafil), a drug that opens (widens) blood vessels, with good response.
However, she still had reduced urine volume and had to undergo dialysis. Treatment for high blood pressure was also continued.
The patient was not a candidate for kidney transplantation due to the progression of her pulmonary hypertension and intolerance to corticosteroids. She continued to suffer from nausea and abdominal pain. Further examination revealed impaired esophageal function, probably a consequence of SSc. The esophagus is the tube that connects the throat to the stomach.
Because of recurrent aspiration events — when food or liquid accidentally enters the airway — treatment with immunosuppressive drugs has been delayed. She was also off disease-modifying therapies.
“Given the complexity of the case, with multiple systems involved, the patient is being followed up by multiple specialty units, including nephrology, rheumatology, respiratory and gastroenterology,” the researchers wrote, adding that “future management decisions will require a multidisciplinary approach.”
