Thrombotic microangiopathy and Purtscher‐like retinopathy associated with adult‐onset Still's disease: A role for glomerular vascular endothelial growth factor?

Adult-Onset Still’s Disease

“…The other rare but quite severe coagulation disorder is thrombotic microangiopathy (TMA). It must be suspected with unexplained multiorgan failure or stroke, related to multiple small thrombi leading to tissue ischemia and mechanical hemolytic anemia [60,74,75]. Acute vision impairment, such as blurred vision related to Purtscher-like retinopathy, is frequently the first symptom [74].…”

“…It must be suspected with unexplained multiorgan failure or stroke, related to multiple small thrombi leading to tissue ischemia and mechanical hemolytic anemia [60,74,75]. Acute vision impairment, such as blurred vision related to Purtscher-like retinopathy, is frequently the first symptom [74]. Key diagnostic tests display thrombocytopenia by platelet consumption, anemia, and schistocytes (fragmented red blood cells), which are specific to this diagnosis.…”

“…In addition, ADAMTS13 enzymatic activity needs to be tested because acquired deficiency has been found to predispose to TMA [59,[96][97][98]. TMA has been mainly described during AoSD flare, related to intense inflammation or to concomitant infection with Shiga toxin-producing microorganisms [74].…”

“…-Renal involvement limited to isolated proteinuria or related to glomerular or interstitial nephritis [102,103]. Acute renal failure is possible in the context of severe myositis, hepatic insufficiency, or hematological complications [21,74,[102][103][104]. -Secondary amyloid A amyloidosis (AA amyloidosis) is nowadays exceptional and is related to sustained uncontrolled inflammation [89][90][91][92][93].…”

Diagnostic and Therapeutic Challenges

“…Purtscher-like retinopathy has been rarely reported in association with adult-onset Still disease. 1,2 Adult-onset Still disease is a diagnosis of exclusion and as such should only be made (by a rheumatologist) after an extensive search for infections, vasculitides, other connective tissue diseases, malignancies, and granulomatous diseases has been concluded. As in this case, aggressive immunosuppression should be instituted in a timely manner.…”