Man’s psoriasis triggers kidney failure, aHUS in rare overlap
Treatment including Soliris helped patient improve, per case report
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A man who developed a severe, full-body flare of psoriasis that caused his skin to peel and swell and triggered kidney failure and atypical hemolytic uremic syndrome (aHUS) improved after being treated with corticosteroids, intravenous immunoglobulin, and the complement inhibitor Soliris (eculizumab).
“Early recognition of this overlap syndrome and prompt complement inhibition are crucial for preventing irreversible kidney disease,” researchers wrote in “Erythrodermic psoriasis complicated by immune complex-mediated crescentic glomerulonephritis and atypical hemolytic uremic syndrome: a case report,” published in BMC Nephrology.
In aHUS, clots form in small blood vessels due to abnormal activity of the complement system (a part of the immune system that helps the body fight infection), causing damage to the kidneys and other organs. aHUS symptoms often appear following a triggering episode, which can be an infection, an autoimmune disease, or another inflammatory stress.
The researchers said the man’s case was an “exceptionally rare” clinical scenario in which erythrodermic psoriasis — a rare form of psoriasis, a skin disease that causes a red rash — overlapped with membranoproliferative glomerulonephritis (MPGN) and aHUS. MPGN is an inflammatory response that affects the filtering units of the kidneys and is associated with complement system activation.
The 52-year-old patient, in China, developed a severe flare of erythrodermic psoriasis. Over three months, more than 90% of his skin reddened and began to peel, a condition known as desquamation. About a month before visiting the hospital, he also developed swelling throughout his body and increasing shortness of breath. He had been taking herbal supplements containing plant extracts for insomnia.
Tests lead doctors to consider several diagnoses
The man had hypertension (high blood pressure) that had been diagnosed two months earlier and was controlled with medications. The skin on his palms and soles was thickened, a condition known as hyperkeratosis, and had painful cracks. He also had severe pitting edema, meaning that pressing on the swollen skin left a dent. His kidney function had been normal.
Blood and urine tests showed that his kidneys had suddenly stopped functioning properly. His blood creatinine, a waste product that increases when kidneys fail, was very high. He also had proteinuria, meaning large amounts of protein were leaking into his urine, and hematuria, meaning blood cells were present in the urine.
Further tests showed microangiopathic hemolysis, a condition in which red blood cells are damaged as they pass through small blood vessels. This caused anemia (low hemoglobin, the protein that carries oxygen in red blood cells), thrombocytopenia (low platelets, which help blood clot), and elevated levels of LDH, an enzyme released when cells are damaged. Fragmented red blood cells were seen under a microscope. Complement protein C3 was low, suggesting an overactive complement system.
A skin biopsy confirmed erythrodermic psoriasis. A kidney biopsy showed glomerulonephritis (inflammation of the kidney’s filtering units) caused by immune complexes, clusters of antibodies and antigens. There were also signs of thrombotic microangiopathy, in which small blood vessels are damaged and blocked by clots.
Doctors considered several possible diagnoses. The combination of kidney failure, anemia with red blood cell destruction, thrombocytopenia, and low C3 strongly suggested aHUS. The final diagnosis was a rare overlap of three conditions: erythrodermic psoriasis, MPGN, and aHUS.
Treatment started with corticosteroids to reduce inflammation and intravenous immunoglobulin, an antibody given through a vein to regulate the immune response. These helped ease erythrodermic psoriasis, but kidney and blood problems continued. The man was then started on Soliris, an antibody that inhibits complement protein C5.
After starting Soliris, the man improved quickly. His platelets returned to normal, and markers of red blood cell damage decreased within three weeks. His kidney function also improved over time, and the amount of protein in his urine decreased. At the time the report was written, he was stable on ongoing treatment with Soliris and a low dose of corticosteroids.
The case “underscores a critical pathogenic link between severe psoriasis-associated systemic inflammation, immune complex deposition, and complement-mediated microangiopathy,” the researchers wrote.
