Adult-onset Still's disease with concurrent thrombotic microangiopathy: Observations from pooled analysis for an uncommon finding.

BACKGROUND: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder that is characterized by quotidian fevers, arthritis, and an evanescent rash. Occurrence of concurrent thrombotic microangiopathy (TMA) in AOSD is rare. The treatment aspects of TMA in AOSD are actively being debated. METHODS: Medline search using MeSH terms and snowballing yielded a total of 29 articles with co-occurrence of AOSD and thrombotic thrombocytopenic purpura (TTP) including our own. Pooled data were synthesized for descriptive analysis. RESULTS: Median age was 35 years with a majority of females (68.96%). A majority of these studies/patients were either Asian (34.48%) or Caucasian (31.03%). Concurrent TMA at the time of AOSD diagnosis was seen in 65.51% patients. Only 3/29 patients had ADAMTS13 level less than 10%, consistent with TTP and 3/29 were diagnosed with hemolytic uremic syndrome (HUS). The remainder were diagnosed clinically. Complication rate was high, and 15/29 (51.72%) patients died or had permanent neurological/renal/vision/gangrenous complications. Median and mean ferritin peak was observed to be higher (7458 and 12 349, respectively) in patients who either died/had partial remission, compared to those who had complete response (3257 and 10 899, respectively), p = .829. CONCLUSIONS: A majority of patients with AOSD-associated TMA either died or had permanent complications. TMA was diagnosed alongside AOSD in 65% patients, while the rest developed TMA during the course of their disease. Blurred vision may precede TMA and could help risk-stratify high-risk AOSD patients clinically. Glycosylated ferritin remains low several weeks to months after disease remission and may be used to monitor severity of disease process. Further studies are necessary to confirm the existing vascular endothelial growth factor hypothesis in AOSD-associated TMA.

Gastric peroral endoscopic pyloromyotomy (G-POEM) in patients with refractory gastroparesis: a review.

Gastric peroral endoscopic pyloromyotomy (G-POEM or POP) is an endoscopic therapeutic modality for treatment of refractory gastroparesis. Since the first case reported in 2013, there are more than 200 papers published on G-POEM. In this narrative review, we summarize the short-term and long-term outcomes and review other important studies. The technical success rate is 100% and the short-term (within 1 year) success rate is about 50-80%. The procedure time is between 50 and 70 min while the average length of hospital stay was 2-3 days. The adverse event rate was around 10%. Few patients need further intervention. Three studies showed that at the 4-year follow-up, the response to G-POEM was durable, but there was a yearly recurrence rate of 13% or more. Redo G-POEM is feasible and can be of benefit for some patients. Most of the studies showed that long duration of illness is associated with poor outcomes. However, reliable predictors for successful outcomes are still unknown. Current literature indicates G-POEM is superior to gastric electric stimulator and surgical pyloroplasty. Endoflip has been used at G-POEM to predict the outcome, but the result is very preliminary. A recent sham study confirms the short-term efficacy of G-POEM. G-POEM is safe and about 50% of patients can be discharged to home on the same day. G-POEM allows for direct biopsy of the gastric muscle, which is the location of the pacemaker cells, the interstitial cells of Cajal; therefore, G-POEM may provide a new path for further research on the pathogenesis of gastroparesis.