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How do I assess PH in connective tissue disease?

Separate SSc screening, disease classification, ILD extent and prognosis after confirmed PAH.

Overview

Open Pulmonary Hypertension → CTD & scleroderma. PAH screening includes ASIG and a DETECT eligibility/referral guide. Enter adult SSc context and the requested values; unknown fields remain unknown. ASIG uses haemoglobin-corrected DLCO, FVC percent predicted and NT-proBNP, not BNP.

The DETECT guide checks eligibility and interprets totals you obtain from the linked published nomogram. It does not calculate DETECT points from raw clinical values. Use the nomogram checklist and its original study restrictions; a negative screen never overrides unexplained symptoms.

SSc classification applies the 2013 ACR/EULAR criteria, including exclusions and category maxima. SSc-ILD extent applies the Goh staging scheme to established SSc-ILD. Neither tool diagnoses PAH, estimates PAH mortality or independently distinguishes group 1 from group 3.

Literature review provides a focused reading guide, organized by clinical question. Choose a topic or search for a drug, trial or finding, then expand a report for its population, result, interpretation, limitations and primary source. Positive, negative/neutral, exploratory, descriptive/prognostic and guideline labels describe different forms of evidence. Subgroups, extensions and general PAH results are not presented as separate SSc trials.

Workup & treatment explains competing mechanisms and next investigations. After RHC-confirmed group 1 PAH, open the existing Risk tools for three-strata, four-strata/COMPERA and REVEAL assessment. Inputs remain in page memory and clear on reload. This prototype requires clinical validation.

Step by step

  1. Choose the clinical question before choosing a tool.
  2. Enter the available findings and inspect missing-data and eligibility messages.
  3. Use the workup pathway and linked primary sources before interpreting an output.

References & further reading

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