Evidence map›Paper›PMID 30874932›Full record

ArticleZeitschrift fur Rheumatologie2019

A case of Hughes-Stovin syndrome (incomplete Behçet's disease) with extensive arterial involvement : Unmasking the true face of a rare syndrome.

Y Emad, Y Ragab, A El-Marakbi, A Saad, O Ibrahim, A Abd-Elhalim, H El-Santawi, J J Rasker

Erratum issuedAbstract readCase Reports
PubMed Publisher
In one paragraph

Article in Zeitschrift fur Rheumatologie, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 11 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed, 1 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

11 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Hughes-Stovin syndrome: An uncommon cause of pulmonary aneurysm and thrombosis.Lung India : official organ of Indian Chest Society · 2026
    Article
  3. Article
  4. Review
  5. Article
  6. Hughes-Stovin Syndrome: An Experience of Management of 3 Cases From a Tertiary Health Care Centre.Clinical medicine insights. Arthritis and musculoskeletal disorders · 2023
    Article
  7. Article
  8. Review
  9. Article
  10. Recurrent Haemoptysis Revealing Hughes-Stovin Syndrome.European journal of case reports in internal medicine · 2021
    Article
  11. Review
4 · The record

Corrections and comments

  • Erratum issued
    2019
5 · Who and what money

Authors and funding

8 authors.

Y EmadRheumatology Department, Faculty of Medicine, Cairo University, Cairo, Egypt. yasseremad68@gmail.com.
Y RagabRadiology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.
A El-MarakbiVascular Surgery Department, Faculty of Medicine, Cairo University, Cairo, Egypt.
A SaadInternal medicine Department, Faculty of Medicine, Cairo University, Cairo, Egypt.
O IbrahimMorecambe Bay University Hospitals Lancaster, Lancashire, UK.
A Abd-ElhalimVascular Surgery Department, Dr.Erfan and Bagedo General Hospital, Jeddah, Saudi Arabia.
H El-SantawiVascular Surgery Department, Dr.Erfan and Bagedo General Hospital, Jeddah, Saudi Arabia.
J J RaskerFaculty of Behavioral, Management and Social Sciences, Department Psychology, Health and Technology, University of Twente, Enschede, The Netherlands.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Hughes-Stovin syndrome (HSS), characterized by the combination of multiple pulmonary artery aneurysms and deep vein thrombosis, is a rare and an under-recognized clinical entity with less than 40 published cases in English medical literature. Vascular venous thrombotic events, as occurring in the course of Behçet's disease (BD), are also described in HSS, e. g., vena cava, intra-cardiac, jugular vein, iliac vein, femoral vein, and dural sinus thrombosis. We describe a 35-year-old man with HSS showing classical features of the syndrome in the form of recurrent thrombophlebitis of the lower limb veins, pulmonary arterial aneurysms, and left lower limb ischemia with extensive arterial tree involvement. The patient presented with critical arterial ischemia in the left lower limb together with aortic and left common iliac artery thrombosis, occlusion of the left superficial femoral artery, and occlusion of both lower limb arteries. Urgent vascular surgeries were carried out for limb salvage. Shortly after, the patient started on pulse corticosteroid/cyclophosphamide therapy, followed by monthly cyclophosphamide for 1 year, with much improvement. We discuss arterial involvement in HSS and similarities of HSS and BD regarding thrombotic events. We summarize the current management options of HSS.

Indexed as

AneurysmBehcet SyndromePulmonary EmbolismVasculitisAdultDiagnosis, DifferentialHumansMalePulmonary ArteryThromboembolismAortitisArterial vasculitis in Hughes–Stovin syndromeHughes–Stovin syndromeIncomplete BehçetPulmonary artery aneurysmsThrombophlebitis

Identifiers

PMID30874932

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.