24 May 2023 : Case report
[In Press] An Initially Missed Diagnosis of Venous Thromboembolic Phenomenon in Adult-Onset Still’s Disease: A Case Report and Literature Review
Unusual clinical course, Mistake in diagnosis, Diagnostic / therapeutic accidents, Management of emergency care, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis), Rare coexistence of disease or pathologyMahlatse Mankgele 1ABCDEFG, Delisa Hlawe 1ABCDEFG, Zayithwa Fabiano1ABCDEFG, Ahmed Solomon2ABCDEFG, Riju Thomas3DF, Mbali Matyeshana4BCDF, Olwethu Mbakaza5DF, Mpoti Seboka2ABCDEFG
Am J Case Rep In Press; DOI: 10.12659/AJCR.939520
Available online: 2023-05-24, In Press, Corrected Proof
Publication in the "In-Press" formula aims at speeding up the public availability of the pending manuscript while waiting for the final publication. The assigned DOI number is active and citable. The availability of the article in the Medline, PubMed and PMC databases as well as Web of Science will be obtained after the final publication according to the journal schedule
Still’s disease is a rare multisystemic autoinflammatory disorder. The diagnosis of adult-onset Still’s disease (AoSD) can be challenging due to the rarity and overlapping features with many other systemic disorders. Complications of the illness can involve many systems in the human body. One of the least documented hematological complications of AoSD is thromboembolic phenomena.
This text outlines the presentation of a 43-year-old woman with a known diagnosis of AoSD, whose disease-modifying anti-rheumatic drugs (DMARDs) had been tapered and stopped due to remission. She presented with respiratory symptoms and features of an AoSD flare. Lack of complete improvement on antibiotic therapy and reinitiating of DMARDs prompted seeking an alternative/concurrent diagnosis. The work-up yielded a pulmonary embolism (PE) on the background of having no other risk factors for thrombosis.
In the reviewed literature, there is a close association between hyperferritinemia and AoSD complicated with venous thromboemboli (VTEs). A rigorous search for alternative diagnoses as well as other potential uncommon complications of AoSD is needed when working-up patients with AoSD, especially those that are not getting better on therapy. Given the rarity of AoSD, meticulous data collection may be useful in understanding the pathophysiology and features of presentation of the illness, including complications such as VTEs.
Keywords: Thrombosis; Still’s Disease, Adult-Onset
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