This rare case was described in the study, “A Case of Anticentromere Antibody-positive Scleroderma Renal Crisis Requiring Dialysis” that was published in the journal Internal Medicine.
The 70-year-old men was admitted to the hospital in Tokyo, Japan, due to kidney dysfunction and hypertension (high blood pressure). Seven months before admission he had normal creatinine levels, a biomarker of kidney function; but since his blood pressure began to rise, his creatinine blood levels also started to increase.
He started taking an antihypertensive agent to manage the blood pressure, but his kidneys continued to show signs of rapid progressive deterioration, with creatinine increasing up to 3.6 times in five months (from 1.04 mg/dL to 3.8 mg/dL).
Two weeks before admission, his creatinine blood levels were more than seven times higher (6.91 mg/dL) than normal range.
At a first evaluation once the patient was admitted to the hospital, he had high blood pressure and skin thickening of the fingers. He had mild skin thickness, as determined by modified Rodman’s skin thickness score (mRSS of 1), and a clinical history of Raynaud’s phenomenon, a condition characterized by poor blood supply to the fingers and toes.
A blood analysis showed he had low levels of red blood cells (anemia), while a urine analysis revealed signs of protein and red blood cell content, both suggestive of renal impairment.
An evaluation of his autoantibodies profile showed that the patient had about a 13 times higher amount of anticentromere antibody, while he was negative for all other tested antibodies. About 60-80% percent of limited SSc patients test positive for anticentromere antibodies.
Additional tests revealed he had interstitial pneumonia, his left kidney was slightly smaller than normal, and he had damage to the eye due to his high blood pressure.
“The presence of Raynaud’s phenomena, skin thickening of the fingers, interstitial lung disease, and positive anticentromere antibody finding indicated limited cutaneous SSc,” the researchers wrote.
Because of his severe hypertension and rapidly progressive kidney failure, he was clinically diagnosed with scleroderma renal crisis (SRC).
Commonly, SRC is diagnosed just by its clinical features not requiring a renal biopsy to be confirmed. However, such an event is extremely rare among patients with anticentromere antibody-positive limited cutaneous SSc, with only a few cases being reported.
Because of its rarity, the clinical team decided to perform a renal biopsy to rule out other kidney diseases.
The results from the biopsy confirmed that his renal crisis was likely caused by scleroderma in combination with his severe hypertension.
Fourteen days after admission, he started hemodialysis due to his deteriorating renal condition. Despite a reduction in blood pressure thanks to antihypertensive therapies, his urine volume continued to decrease, and hemodialysis could not be withdrawn.
“To our knowledge, this is the first report to describe the [clinical characteristics] of SRC in a patient with anticentromere antibody-positive limited cutaneous SSc,” the researchers wrote.
According to the team, although antihypertensive therapies, specifically ACE inhibitors, are commonly used to manage hypertension, “the renal prognosis [of these rare cases] may be poor.”
The researchers emphasize that additional analyses of these rare cases are needed to have a better understanding of the clinical course and prognosis of scleroderma renal crisis in patients with anticentromere antibody-positive limited cutaneous SSc.