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ORPHA:188

Systemic capillary leak syndrome

low confidenceDisorder

Also known as: Capillary hyperpermeability syndrome · Capillary leak syndrome · Clarkson disease · Idiopathic capillary leak syndrome · SCLS

Publications

4,600

Trials

0

Interventional, condition-specific

Researchers

947

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

Systemic capillary leak syndrome (SCLS) is a severe systemic disease due to increased capillary permeability, characterized by episodes of hypotension, edema and hypovolemia.

How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (8)

AVLS · CLS · Systemic Capillary Leak Syndrome · acute vascular leak syndrome · capillary hyperpermeability syndrome · capillary leak syndrome · idiopathic capillary leak syndrome · systemic capillary leak syndrome

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

2/6 stages with a signal

Research-stage checklist from open sources (GenCC, literature, Monarch when enriched, ClinicalTrials.gov). Not a prognosis or care recommendation.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    4,600 matched papers (2,829 in last 10 years) Source

  3. Phenotype characterisedPresent

    23 HPO annotations (e.g. Pancreatitis; Pedal edema; Weight loss) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialNot found

    No matched interventional trial under our ClinicalTrials.gov rules

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

23

Associated phenotypes · MONDO:0001956

  • Pancreatitis
  • Pedal edema
  • Weight loss
  • Diarrhea
  • Fatigue

Showing 5 of 23 — open Monarch for the full list.

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

16 associated chemicals. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Aminophylline · therapeutic
  • Curcumin · therapeutic
  • Dexamethasone · therapeutic
  • Glucocorticoids · therapeutic
  • Hydroxyethyl Starch Derivatives · therapeutic
  • Terbutaline · therapeutic
  • VAD combination · therapeutic
  • Acitretin · marker/mechanism
  • aldesleukin · marker/mechanism
  • Bortezomib · marker/mechanism
  • Cisplatin · marker/mechanism
  • Clofarabine · marker/mechanism

MyDisease.info · MONDO:0001956

Literature

Is anyone studying this?

4,600

4,600 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

4,600 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

2,829 in the last 10 years · low confidence

Phrase hits: 4,600 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

947

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.

  1. 01
    Druey KM20 papers · 2024

    Laboratory of Allergic Diseases, NIAID/NIH, Bethesda, MD, USA.

    Papers in Europe PMC
  2. 02
    Xie Z11 papers · 2019

    Laboratory of Allergic Diseases, NIAID/NIH, Bethesda, MD, USA.

    Papers in Europe PMC
  3. 03
    Amoura Z8 papers · 2022

    Service de médecine interne 2, CHU La Pitié-Salpêtrière, APHP, Université Paris 6, France. Electronic address: zahir.amoura@aphp.fr.

    Papers in Europe PMC
  4. 04
    Parikh SM8 papers · 2024

    Department of Medicine, Division of Nephrology and Center for Vascular Biology Research, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, USA.

    Papers in Europe PMC
  5. 05
    Pineton de Chambrun M8 papers · 2022

    1Service de Médecine Interne 2, Centre Hospitalier Universitaire (CHU) La Pitié-Salpêtrière, Université Paris 6, APHP, Paris, France.2Service de Réanimation Médicale, CHU La Pitié-Salpêtrière, Université Paris 6, APHP, Paris, France.3Service de Réanimation Médicale, CHU d'Angers, Angers, France.4Service de Médecine Interne, CH Bretagne Atlantique, Vannes, France.5Service de Réanimation Médicale, CHU Edouard-Herriot, Lyon, France.6Service de Réanimation, CHU Lyon-Sud, Pierre-Bénite, France.7Service de Réanimation Polyvalente, CH Pierre Oudot, Bourgoin Jallieu, France.8Service de Réanimation Médicale, CHRU Lille, Lille, France.9Service de Réanimation Médicale, CHU Bichat, APHP, Paris, France.10Service de Réanimation Polyvalente, Hôpital Delafontaine, Saint-Denis, France.11Service de Réanimation Polyvalente, CH André-Mignot, Le Chesnay, France.12Service de Réanimation Médicale, CHU Hôtel-Dieu, Nantes, France.13Service de Réanimation Médicale, CHU Henri-Mondor, APHP, Créteil, France.14Service de Pneumologie et de Réanimation Médicale, CHU La Pitié-Salpêtrière, APHP, Paris, France.15Service de Réanimation Chirurgicale, CHU Lyon-Est, Hôpital Louis-Pradel, Bron, France.16Service de Réanimation Médicale, Hôpital Gabriel-Montpied, CHU Clermont-Ferrand, Clermont-Ferrand, France.17Service de Réanimation Polyvalente, CH Notre-Dame de la Miséricorde, Ajaccio, France.18Service de Réanimation Médicale, CHU Ambroise-Paré, APHP, Boulogne-Billancourt, France.19Service de Réanimation Médicale, CHU Grenoble, Grenoble, France.20Service de Réanimation Médicale, CHU Saint-Louis, APHP, Paris, France.21Service de Réanimation, Hôpital du Valais-Institut Central, Sion, Switzerland.22Service de Réanimation, Hôpital d'Instruction des Armées Desgenettes, Lyon, France.23Service de Réanimation Polyvalente, CH Foch, Suresnes, France.24Service de Réanimation, CH Dax, Dax, France.25Service de Réanimation Polyvalente, CH Bretagne Atlantique, Vannes, France.26Service de Réanimation Médicale, CHU Cochin, APHP, Paris, France.27Service de Réanimation Adulte, CHU Necker-Enfants Malades, APHP, Paris, France.

    Papers in Europe PMC
  6. 06
    Colombo R7 papers · 2025

    Division of Anesthesiology and Intensive Care, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy.

    Papers in Europe PMC
  7. 07
    Wu MA7 papers · 2025

    Division of Internal Medicine, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan, Via G.B. Grassi 74, 20157, Milan, Italy. maddalena.wu@unimi.it.

    Papers in Europe PMC
  8. 08
    Chan EC6 papers · 2024

    Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases/National Institutes of Health, Bethesda, Md.

    Papers in Europe PMC
  9. 09
    Long LM6 papers · 2019

    National Institute of Allergy and Infectious Diseases, National Institutes of Health, 50 South Drive Room 4154, Bethesda, MD, 20892-8305, USA.

    Papers in Europe PMC
  10. 10
    Luyt CE6 papers · 2022

    Service de Réanimation Médicale, Institut de Cardiologie, Hôpital La Pitié-Salpêtrière, Sorbonne Université, Assistance Publique Hôpitaux de Paris, Paris, France.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

0

interventional trials for this specific condition

No interventional trial testing a treatment matched this specific condition name. 4 observational studies did — shown below because natural-history and cohort work can be an important step toward a trial.

Data as of 11 September 2026 · last trial check 11 September 2026

No matched interventional trials. This is true for 77.2% of diseases in the trials denominator (5501 of 7126). Here are the researchers publishing on it.

low confidence · 38.6th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.

See who's working on it — people publishing on this disease are often the practical next contact when no trial is listed.

Observational and natural-history studies

4 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

None of the matched observational studies is currently listed as recruiting.

Open the complete matched search on ClinicalTrials.gov

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 16 · after dedupe 16 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 16 · dropped 0 · fetched 2026-07-29

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (16)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Systemic capillary leak syndrome — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

("Systemic capillary leak syndrome" OR "Capillary hyperpermeability syndrome" OR "Capillary leak syndrome" OR "Clarkson disease" OR "Idiopathic capillary leak syndrome" OR "acute vascular leak syndrome")

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Systemic capillary leak syndrome" OR "Capillary hyperpermeability syndrome" OR "Capillary leak syndrome" OR "Clarkson disease" OR "Idiopathic capillary leak syndrome" OR "acute vascular leak syndrome"

Study-type breakdown: 0 interventional · 4 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: SCLS; AVLS; CLS

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 3 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus
  • Publication count (4600) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity

Ingested 2026-07-26T12:49:57.302Z