{"schema":"https://ver.cy/schemas/card/1.0.0","id":"vr.tr.hughes-stovin-syndrome","code":"thing-q609675","url":"https://ver.cy/models/thing/q609675/","name":"Hughes-Stovin syndrome","alternateNames":[],"kind":"thing","status":"research-draft","version":"0.1.0-research.1","language":"en","classifiers":{"family":"Thing Registry","category":"Information and virtual systems","entryKind":"thing","plane":"INF","domain":["INF.KNW"],"industry":[],"navPath":"","tags":[],"facets":{}},"whatItIs":"Hughes-Stovin syndrome is a very rare vascular inflammatory syndrome characterized by thrombophlebitis and pulmonary arterial aneurysms, sometimes with bronchial arterial involvement, whose relationship to Behçet disease remains unresolved.","purpose":"Enable an agent to recognise the Hughes-Stovin syndrome phenotype, record diagnostic uncertainty and vascular disease state, and support specialist decisions involving competing thrombosis and haemorrhage risks.","scope":{"in":["Association of pulmonary arterial aneurysmal disease with venous thrombosis and thrombophlebitis.","Diagnostic evidence, alternative explanations and overlap with Behçet disease.","Lesion-specific pulmonary vascular anatomy, thrombosis and bleeding status.","Temporal relationships among inflammatory activity, thrombosis, aneurysm development and haemorrhage.","Disease-specific constraints on immunosuppression, antithrombotic treatment and vascular intervention.","Versioned diagnostic criteria, terminology mappings and limits of epidemiological evidence."],"out":["The patient's complete demographic, medical and social record.","The encounter, referral, admission or episode of care itself.","Comprehensive models of Behçet disease, pulmonary embolism or inherited thrombophilia.","Generic imaging acquisition protocols and radiology equipment specifications.","Drug formularies, dosing protocols and procedural technique manuals."],"boundaries":[]},"distinguishingFeatures":["Look for the combination of pulmonary artery aneurysms and venous thrombosis; either feature alone is insufficient to establish this syndrome. [HSSISG vascular study](https://pubmed.ncbi.nlm.nih.gov/33529654/)","Assess pulmonary arterial thrombus in its vessel-wall and aneurysm context rather than automatically labelling every filling defect as embolic disease. [HSSISG imaging atlas](https://pmc.ncbi.nlm.nih.gov/articles/PMC8599253/)","Evaluate Behçet manifestations and the criteria used to assess them; absent mucocutaneous findings alone should not be treated as definitive proof of a separate disease.","Require a documented differential assessment of infectious, traumatic, iatrogenic and other causes of pulmonary aneurysmal disease before assigning the syndrome label.","Distinguish preliminary criteria from validated diagnostic standards: the HSSISG has proposed preliminary criteria whose exact version and validation status must accompany their use. [HSSISG preliminary criteria report](https://pubmed.ncbi.nlm.nih.gov/41984348/)"],"structure":{"bundles":[{"id":"syndrome-attribution","name":"Syndrome attribution","description":"Represent why a vascular phenotype is attributed to Hughes-Stovin syndrome and how secure that attribution is.","layers":[{"id":"defining-vascular-association","name":"Defining vascular association","description":"Connect pulmonary aneurysmal disease with independently documented thrombotic manifestations.","findings":[{"id":"phenotype-evidence","name":"Phenotype evidence","description":"Record the evidence and dates supporting each component, including components not yet assessed.","questions":[{"text":"Which pulmonary vascular lesions and venous thrombotic events support the proposed syndrome attribution?","id":"phenotype-evidence-q01","kind":"definition"},{"text":"Which imaging reports, clinical observations and assessors establish each component?","id":"phenotype-evidence-q02","kind":"provenance"}]}]},{"id":"criteria-and-coding","name":"Criteria and coding","description":"Attach explicit versions and evidential limits to diagnostic frameworks and terminology mappings.","findings":[{"id":"versioned-attribution","name":"Versioned attribution","description":"Record the applied framework, its issuing body and status, and separately verified coding mappings.","questions":[{"text":"Was attribution based on clinical judgement or specified HSSISG preliminary criteria, and which publication and version were used?","id":"versioned-attribution-q01","kind":"provenance"},{"text":"Which ICD-10, ICD-11 or SNOMED CT release supports the recorded mapping, and is it exact or broader?","id":"versioned-attribution-q02","kind":"boundary"}]}]}]},{"id":"behcet-and-mimic-boundaries","name":"Behçet and mimic boundaries","description":"Represent overlap with Behçet disease and competing explanations for the vascular findings.","layers":[{"id":"behcet-overlap","name":"Behçet overlap","description":"Preserve the findings and reasoning used to assess a Behçet-spectrum interpretation.","findings":[{"id":"overlap-evidence","name":"Overlap evidence","description":"Record mucosal, ocular, cutaneous and other relevant manifestations with explicit distinction between absence and lack of assessment.","questions":[{"text":"Which Behçet-associated manifestations have been assessed, and which are present, absent or unknown?","id":"overlap-evidence-q01","kind":"measurement"},{"text":"What supports retaining the Hughes-Stovin label, recording overlap or revising attribution to Behçet disease?","id":"overlap-evidence-q02","kind":"boundary"}]}]},{"id":"alternative-causes","name":"Alternative causes","description":"Track competing explanations for aneurysms and thrombosis without requiring an unsupported universal exclusion panel.","findings":[{"id":"differential-resolution","name":"Differential resolution","description":"Record investigation of infection, vascular injury, structural vascular disease and alternative thrombotic mechanisms as clinically relevant.","questions":[{"text":"Which alternative causes could explain the pulmonary vascular lesions or the thrombotic distribution?","id":"differential-resolution-q01","kind":"boundary"},{"text":"What evidence supports, weakens or leaves unresolved each competing explanation?","id":"differential-resolution-q02","kind":"provenance"}]}]}]},{"id":"pulmonary-lesions-and-haemorrhage","name":"Pulmonary lesions and haemorrhage","description":"Represent pulmonary and bronchial vascular lesions and their relationship to bleeding.","layers":[{"id":"lesion-anatomy","name":"Lesion anatomy","description":"Maintain identifiable lesions with comparable measurements and radiological interpretations.","findings":[{"id":"aneurysm-and-thrombus-map","name":"Aneurysm and thrombus map","description":"Separate artery, lesion morphology, wall findings and associated thrombus; the HSSISG atlas supplies a disease-specific imaging reference. [HSSISG imaging atlas](https://pmc.ncbi.nlm.nih.gov/articles/PMC8599253/)","questions":[{"text":"Where is each lesion, what are its dimensions, and is it interpreted as a true aneurysm, pseudoaneurysm or indeterminate lesion?","id":"aneurysm-and-thrombus-map-q01","kind":"measurement"},{"text":"What wall and thrombus findings were observed, and how certain is the interpretation of in situ thrombosis versus embolism?","id":"aneurysm-and-thrombus-map-q02","kind":"measurement"}]}]},{"id":"bleeding-instability","name":"Bleeding instability","description":"Link clinical bleeding to suspected vascular sources and radiological evidence of instability.","findings":[{"id":"haemorrhage-assessment","name":"Haemorrhage assessment","description":"Record haemoptysis, physiological consequences and suspected leakage or rupture without assuming every episode has the same source.","questions":[{"text":"Is haemoptysis active, what is its physiological impact, and which lesion is suspected to be responsible?","id":"haemorrhage-assessment-q01","kind":"measurement"},{"text":"Do the clinical or imaging findings require immediate emergency assessment or specialist vascular intervention review?","id":"haemorrhage-assessment-q02","kind":"action"}]}]}]},{"id":"vascular-course-and-activity","name":"Vascular course and activity","description":"Track thrombotic extent, pulmonary lesion evolution and evidence of inflammatory activity over time.","layers":[{"id":"thrombotic-course","name":"Thrombotic course","description":"Link individual thrombotic events across vascular territories and dates.","findings":[{"id":"distribution-and-recurrence","name":"Distribution and recurrence","description":"Record event location, confirmation method, extension, resolution and recurrence.","questions":[{"text":"Which thrombi are newly detected, extending, persistent, resolved or recurrent, and how was each status established?","id":"distribution-and-recurrence-q01","kind":"measurement"},{"text":"Did the thrombotic events precede, coincide with or follow recognition of pulmonary aneurysmal disease?","id":"distribution-and-recurrence-q02","kind":"provenance"}]}]},{"id":"activity-versus-damage","name":"Activity versus damage","description":"Distinguish current inflammatory evidence from persistent vascular abnormalities and their consequences.","findings":[{"id":"longitudinal-state","name":"Longitudinal state","description":"Maintain dated observations and explicit clinician interpretations rather than imposing an unverified universal staging system.","questions":[{"text":"What supports active vascular inflammation, improvement, relapse or persistent structural damage at this assessment?","id":"longitudinal-state-q01","kind":"measurement"},{"text":"Are apparent changes supported by comparable imaging and measurements, or could technique differences explain them?","id":"longitudinal-state-q02","kind":"boundary"}]}]}]},{"id":"treatment-decisions-and-evidence","name":"Treatment decisions and evidence","description":"Capture disease-specific treatment reasoning, response and uncertainty without prescribing a universal regimen.","layers":[{"id":"competing-treatment-risks","name":"Competing treatment risks","description":"Record the specialist assessment connecting vascular state to treatment choices.","findings":[{"id":"individualised-treatment-rationale","name":"Individualised treatment rationale","description":"Link decisions about immunosuppression, antithrombotic therapy and intervention to documented indications, bleeding assessment and responsible clinicians.","questions":[{"text":"What disease activity, thrombotic indication and aneurysm or bleeding assessment support the selected treatment approach?","id":"individualised-treatment-rationale-q01","kind":"action"},{"text":"Who reviewed the competing risks, what uncertainty remains, and what findings would trigger reconsideration?","id":"individualised-treatment-rationale-q02","kind":"provenance"}]}]},{"id":"response-and-generalisability","name":"Response and generalisability","description":"Separate observed outcomes from causal conclusions and population-level claims.","findings":[{"id":"outcome-evidence","name":"Outcome evidence","description":"Record lesion response, recurrent thrombosis, bleeding and treatment harms while identifying the evidence population and follow-up period.","questions":[{"text":"After treatment, what changed in identified aneurysms, thrombi, haemoptysis and inflammatory observations, and over what interval?","id":"outcome-evidence-q01","kind":"measurement"},{"text":"Does a prognostic or treatment claim derive from an individual case, selected case series or population study, and what limits its applicability?","id":"outcome-evidence-q02","kind":"provenance"}]}]}]},{"id":"evidence-and-external-alignment","name":"Evidence and external alignment","description":"What the world already says about this thing, gathered so the model can be checked against it.","layers":[{"id":"reported-evidence","name":"Reported evidence","description":"Findings from the breadth pass, kept separate from the structural claims.","findings":[{"id":"evidence-confidence-notes","name":"Check these first","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these check these first hold for the sense of Hughes-Stovin syndrome this model covers, and on what evidence?","id":"evidence-confidence-notes-q01","kind":"provenance"}]},{"id":"evidence-real-world-use","name":"Real-world use","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these real-world use hold for the sense of Hughes-Stovin syndrome this model covers, and on what evidence?","id":"evidence-real-world-use-q01","kind":"provenance"}]},{"id":"evidence-failure-modes-and-hazards","name":"Failure modes and hazards","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these failure modes and hazards hold for the sense of Hughes-Stovin syndrome this model covers, and on what evidence?","id":"evidence-failure-modes-and-hazards-q01","kind":"provenance"}]},{"id":"evidence-neighbours","name":"Neighbouring kinds and how to tell them apart","description":"Recalled without web access and unsourced; every item is a lead to verify.","questions":[{"text":"Which of these neighbouring kinds and how to tell them apart hold for the sense of Hughes-Stovin syndrome this model covers, and on what evidence?","id":"evidence-neighbours-q01","kind":"provenance"}]}]}]}]},"agentConduct":{"may":["Explain Hughes-Stovin syndrome and its possible relation to Behcet disease, citing medical sources.","Advise anyone coughing up blood to seek urgent care."],"mustNot":["Diagnose the syndrome from symptoms or images.","Advise anticoagulation or immune treatment for a person.","Delay urgent care for coughing up blood.","Share a person's rare diagnosis in a way that identifies them."],"requiresHuman":["Any clinical decision about the treatment of a person."]},"ethics":{"considerations":["Aneurysm rupture can be fatal; treatment choices carry real danger.","Rare diseases make patients easier to identify and harder to diagnose."],"affectedParties":["Patients","Families of patients","Clinicians"]},"owners":{"steward":"Nobody owns the condition; clinicians answer for each patient's care.","roles":[],"masterSystems":[]},"relations":[{"target":"Q55785504","type":"parent","note":"registry parent class"}],"interaction":{"identity":{"applicability":"required","items":["Vercy registry: vr.tr.hughes-stovin-syndrome","Wikidata: Q609675 (https://www.wikidata.org/wiki/Q609675)"]},"properties":{"applicability":"not-applicable","items":["Diagnostic attribution: suspected | clinician-attributed | disputed | revised; assessor and date","Diagnostic framework: issuing body, publication, version, assessed items and validation status","Pulmonary vascular lesion phenotype: artery and segment; aneurysm | pseudoaneurysm | other | indeterminate; imaging interpretation","Pulmonary aneurysm dimensions: mm, with measurement plane, modality, lesion identifier and date","Thrombotic distribution: linked superficial venous, deep venous, cerebral venous, intracardiac or arterial lesions","Pulmonary arterial thrombus interpretation: in situ favoured | embolic favoured | mixed | indeterminate","Haemoptysis and physiological impact: absent | historical | active; estimated bleeding, oxygenation and haemodynamic consequences","Inflammatory activity evidence: dated symptoms, laboratory results with units and vascular imaging observations","Behçet overlap assessment: manifestations present, absent or unassessed; criteria version; specialist interpretation","Terminology mapping: coding system, release, code, preferred term and exact or broader mapping"]},"recognition":{"applicability":"optional","items":[]},"capabilities":{"applicability":"required","items":["Assemble a dated evidence profile for specialist review of suspected Hughes-Stovin syndrome.","Compare serial imaging of identified aneurysms and thrombi and flag new lesions or deterioration.","Flag active haemoptysis or suspected vascular leakage for urgent clinical escalation.","Expose unresolved haemorrhage and thrombosis considerations before a clinician makes an antithrombotic decision.","Track clinician-selected treatment, lesion response, recurrence and adverse outcomes.","Reassess diagnostic attribution when Behçet manifestations, alternative causes or revised criteria emerge."]},"hazards":{"applicability":"required","items":[]},"interfaces":{"applicability":"required","items":[]},"context":{"applicability":"required","items":[]}},"sources":[{"title":"Wikidata item Q609675: Hughes-Stovin syndrome","url":"https://www.wikidata.org/wiki/Q609675","note":"identity and sense of the item"},{"title":"Wikipedia: Hughes–Stovin syndrome","url":"https://en.wikipedia.org/wiki/Hughes%E2%80%93Stovin_syndrome","note":"general description of the item"}],"openQuestions":["What are the exact items, thresholds, intended population and external validation results of the HSSISG preliminary diagnostic criteria?","Which authoritative ICD-10, ICD-11 and SNOMED CT releases provide an exact Hughes-Stovin syndrome mapping, if any?","Is Hughes-Stovin syndrome best treated as a distinct disease or a Behçet-spectrum phenotype, and what evidence can resolve that boundary?","What population-based prevalence or incidence estimates exist with explicit geography, ascertainment method and year, rather than counts of published cases?","Which imaging features, surveillance intervals and treatment strategies reliably predict or reduce rupture, recurrent thrombosis and relapse, including when antithrombotic therapy is justified?"],"resources":{"spec":"/models/things/publications/thing-q609675/spec.json"},"provenance":{"origin":"thing registry research (pass 2)","builtFrom":["models/things/publications/thing-q609675/spec.json"],"providers":["Codex"],"researchStatus":"unreviewed","generatedAt":"2026-09-12T10:20:33Z","builder":"tools/build_cards.py@1.0.0"},"completeness":{"sections":{"classifiers":"filled","whatItIs":"filled","purpose":"filled","distinguishingFeatures":"filled","structure":"filled","agentConduct":"filled","ethics":"filled","owners":"filled","relations":"filled","interaction.identity":"filled","interaction.properties":"not-applicable","interaction.recognition":"missing","interaction.capabilities":"filled","interaction.hazards":"missing","interaction.interfaces":"missing","interaction.context":"missing","sources":"filled"},"notes":{"interaction.properties":"Plane INF: no invented physical properties."},"score":0.75}}