Thrombotic Thrombocytopenic Purpura and Adult Onset Still’s Disease

Thrombotic microangiopathy in a patient with adult‐onset Still's disease

“…The most recent literature review was published in 2009 and included a total of 15 cases . An additional five cases have been published since this review 8‐12. Our patient's clinical presentation was similar to what had been previously reported.…”

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

“…Various histopathologic features of kidney involvement have been reported in AOSD, including AA amyloidosis, interstitial nephritis, or collapsing glomerulopathy (1). The association of AOSD with TMA is rare, and only 13 cases have been previously reported in the English literature (5–16) (Table 1). In most cases, TMA occurred during an AOSD flare, and AOSD treatment was associated with TMA resolution, which argues for a pathophysiologic link between AOSD and TMA.…”

“…Multiple mechanisms may be involved in TMA pathogenesis (decrease of ADAMTS‐13 activity, complement regulatory proteins dysfunction, or direct endothelial toxicity due to infections by Shiga toxin‐producing microorganisms), but remain unclear in AOSD‐associated TMA. In 2 patients, decreased ADAMTS‐13 activity related to autoantibodies against this protease was reported (14, 16) (Table 1). In our patient, complement regulatory gene mutations or Shiga toxin‐producing microorganism infection were absent, and ADAMTS‐13 activity was only moderately decreased, well above the severe deficiency level (<5%) associated with TMA (3).…”

Adult-Onset Still’s Disease

“…Organ imaging may reveal multiple infarctions. 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].…”