Hughes-Stovin syndrome (HSS) preliminary diagnostic criteria: a report by the HSS International Study Group (HSSISG).
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Emad Yasser, Gheita Tamer, Ragab Yasser, Hammam Nevin, Kindermann Michael, Ibrahim Ossama, Fabi Marianna, Frikha Faten, Alhusseiny Khalid, Tekavec-Trkanjec Jasna, Kechida Melek, Farber Harrison W, Young Pablo, Pankl Sonia, Robinson Cal, Tharwat Samar, Guffroy Aurelien, Jaramillo Natalia, Ruffer Nikolas, Bawaskar Parag, Kably Issam, Abou-Zeid Alaa, Hassan Maged, Ghirardo Sergio, Barman Bhupen, Bennji Sami, Agarwala Manoj Kumar, Tornes Leticia, Margolesky Jason, Silva Rafael S, Cruz Vitor, Abdelbary Mohamed H, Kim Jung Tae, Cozzi Diletta, Abdelali Mabrouk, Joy Tubig C, Sherwina Juljani, Kassem Iman A, Yasser Farida, Ten Klooster Peter M, Rasker Johannes J
Аннотация
METHODS: This international initiative included a systematic literature review of HSS case reports in a global endeavor. A systematic review of MEDLINE, EMBASE, and the Cochrane database identified 112 HSS patients. The selected reports were based on comprehensive case presentations, encompassing demographic characteristics, initial disease presentations, notable symptoms and signs, laboratory findings, radiological investigations, medical lines of treatment or interventional management received, and finally patients' outcomes. Diagnostic criteria were selected based on their clinical relevance, dominance, and consensus among the HSSISG members. Each criterion was discussed in detail to enhance comprehension and interpretation by physicians.
RESULTS: Following consensus agreement, one mandatory entry criterion was selected which included pulmonary findings, along with one exclusion criterion regarding ocular aspects. Two major criteria were defined, vascular aspects and coagulation profile, and four minor criteria: mucocutaneous, central nervous system, cardiac, and inflammatory markers. The maximum achievable score is 30, with a suggested definitive diagnosis of HSS with scores 10 or higher, probable HSS with scores of 5-9, and possible HSS for scores 3-4. Scores < 3 are considered nondiagnostic.
CONCLUSION: Preliminary HSS diagnostic criteria were proposed after a comprehensive and critical literature review by the HSSISG to offer support for early diagnosis and optimal management.