Hughes-Stovin syndrome

Phenotype evidence

Record the evidence and dates supporting each component, including components not yet assessed.

1. Which pulmonary vascular lesions and venous thrombotic events support the proposed syndrome attribution? definition
2. Which imaging reports, clinical observations and assessors establish each component? provenance

Versioned attribution

Record the applied framework, its issuing body and status, and separately verified coding mappings.

1. Was attribution based on clinical judgement or specified HSSISG preliminary criteria, and which publication and version were used? provenance
2. Which ICD-10, ICD-11 or SNOMED CT release supports the recorded mapping, and is it exact or broader? boundary

Overlap evidence

Record mucosal, ocular, cutaneous and other relevant manifestations with explicit distinction between absence and lack of assessment.

1. Which Behçet-associated manifestations have been assessed, and which are present, absent or unknown? measurement
2. What supports retaining the Hughes-Stovin label, recording overlap or revising attribution to Behçet disease? boundary

Differential resolution

Record investigation of infection, vascular injury, structural vascular disease and alternative thrombotic mechanisms as clinically relevant.

1. Which alternative causes could explain the pulmonary vascular lesions or the thrombotic distribution? boundary
2. What evidence supports, weakens or leaves unresolved each competing explanation? provenance

Aneurysm and thrombus map

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/)

1. Where is each lesion, what are its dimensions, and is it interpreted as a true aneurysm, pseudoaneurysm or indeterminate lesion? measurement
2. What wall and thrombus findings were observed, and how certain is the interpretation of in situ thrombosis versus embolism? measurement

Haemorrhage assessment

Record haemoptysis, physiological consequences and suspected leakage or rupture without assuming every episode has the same source.

1. Is haemoptysis active, what is its physiological impact, and which lesion is suspected to be responsible? measurement
2. Do the clinical or imaging findings require immediate emergency assessment or specialist vascular intervention review? action

Distribution and recurrence

Record event location, confirmation method, extension, resolution and recurrence.

1. Which thrombi are newly detected, extending, persistent, resolved or recurrent, and how was each status established? measurement
2. Did the thrombotic events precede, coincide with or follow recognition of pulmonary aneurysmal disease? provenance

Longitudinal state

Maintain dated observations and explicit clinician interpretations rather than imposing an unverified universal staging system.

1. What supports active vascular inflammation, improvement, relapse or persistent structural damage at this assessment? measurement
2. Are apparent changes supported by comparable imaging and measurements, or could technique differences explain them? boundary

Individualised treatment rationale

Link decisions about immunosuppression, antithrombotic therapy and intervention to documented indications, bleeding assessment and responsible clinicians.

1. What disease activity, thrombotic indication and aneurysm or bleeding assessment support the selected treatment approach? action
2. Who reviewed the competing risks, what uncertainty remains, and what findings would trigger reconsideration? provenance

Outcome evidence

Record lesion response, recurrent thrombosis, bleeding and treatment harms while identifying the evidence population and follow-up period.

1. After treatment, what changed in identified aneurysms, thrombi, haemoptysis and inflammatory observations, and over what interval? measurement
2. Does a prognostic or treatment claim derive from an individual case, selected case series or population study, and what limits its applicability? provenance

Check these first

Recalled without web access and unsourced; every item is a lead to verify.

1. Which of these check these first hold for the sense of Hughes-Stovin syndrome this model covers, and on what evidence? provenance

Real-world use

Recalled without web access and unsourced; every item is a lead to verify.

1. Which of these real-world use hold for the sense of Hughes-Stovin syndrome this model covers, and on what evidence? provenance

Failure modes and hazards

Recalled without web access and unsourced; every item is a lead to verify.

1. Which of these failure modes and hazards hold for the sense of Hughes-Stovin syndrome this model covers, and on what evidence? provenance

Neighbouring kinds and how to tell them apart

Recalled without web access and unsourced; every item is a lead to verify.

1. 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? provenance