Patient with ankylosing spondylitis and scleroderma renal crisis

We report a 56-year-old man with a history of ankylosing spondylitis and systemic scleroderma. The patient had been diagnosed with ankylosing spondylitis 20 years ago and had been receiving treatment with NSAIDs and anti TNFα drugs. He referred to our rheumatology department for Raynaud’s phenomenon, arthralgias and weight loss. Physical examination revealed stiffness of the skin with difficulty in pinching (mainly at lower extremities, from knee to ankle). Soon after his first visit to our department, he developed renal scleroderma crisis with abrupt increase in blood pressure, decline in renal function, and microangiopathic haemolytic anaemia in accordance with positive antinuclear autoantibodies and positive anti-topoisomerase I antibody (anti-Scl70). This is one of the few reports in the literature of coexistence of ankylosing spondylitis and systemic scleroderma. A genetic correlation seems to be an explanation in some patients who carry one or two susceptibility alleles to both diseases. Thus, this might be the case of a ‘genetic trap’ in which distinct genes are cooperating to favour the susceptibility to two different HLA-associated systemic autoimmune diseases.