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

Anil Ananthaneni; Gaelen Shimkus; Francesca Weis; Eunice Adu-Dapaah; Rachaita Lakra; Poornima Ramadas; Samina Hayat

doi:10.1111/ejh.14142

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

Eur J Haematol. 2024 Apr;112(4):484-492. doi: 10.1111/ejh.14142. Epub 2023 Nov 23.

Authors

Anil Ananthaneni¹, Gaelen Shimkus², Francesca Weis², Eunice Adu-Dapaah³, Rachaita Lakra², Poornima Ramadas¹, Samina Hayat⁴

Affiliations

¹ Division of Hematology & Oncology, Department of Internal Medicine, Louisiana State University Health Sciences Center, Shreveport, Louisiana, USA.
² Department of Internal Medicine, Louisiana State University Health Sciences Center, Shreveport, Louisiana, USA.
³ Division of Rheumatology, Department of Internal Medicine, University of California, Los Angeles, California, USA.
⁴ Division of Rheumatology, Department of Internal Medicine, Louisiana State University Health Sciences Center, Shreveport, Louisiana, USA.

PMID: 37997494
DOI: 10.1111/ejh.14142

Abstract

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.

Keywords: AOSD; Still's disease; TMA; TTP; aHUS; atypical hemolytic uremic syndrome; thrombotic microangiopathy; thrombotic thrombocytopenic purpura.

Publication types

Review

MeSH terms

Adult
Female
Hemolytic-Uremic Syndrome* / complications
Hemolytic-Uremic Syndrome* / diagnosis
Hemolytic-Uremic Syndrome* / therapy
Humans
Purpura, Thrombotic Thrombocytopenic* / complications
Purpura, Thrombotic Thrombocytopenic* / diagnosis
Still's Disease, Adult-Onset* / complications
Still's Disease, Adult-Onset* / diagnosis
Still's Disease, Adult-Onset* / therapy
Thrombotic Microangiopathies* / complications
Thrombotic Microangiopathies* / diagnosis
Vascular Endothelial Growth Factor A

Substances

Vascular Endothelial Growth Factor A