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| name: Wissler syndrome | ||
| category: Immune | ||
| description: >- | ||
| A rheumatic syndrome of possibly allergic origin, usually affecting children | ||
| and adolescents, and characterized by high fever, exanthema, arthralgia, | ||
| leukocytosis, and increased sedimentation rate. Also called Wissler-Fanconi | ||
| syndrome or subsepsis allergica. | ||
| disease_term: | ||
| term: | ||
| id: MONDO:0006018 | ||
| label: Wissler syndrome | ||
| preferred_term: Wissler syndrome | ||
| parents: | ||
| - Autoinflammatory Disease | ||
| - Inflammatory Arthritis | ||
| synonyms: | ||
| - Wissler-Fanconi syndrome | ||
| - Subsepsis allergica | ||
| - Subsepsis hyperergica | ||
| phenotypes: | ||
| - name: Recurrent high fever | ||
| category: Systemic | ||
| frequency: FREQUENT | ||
| phenotype_term: | ||
| preferred_term: Fever | ||
| term: | ||
| id: HP:0001945 | ||
| label: Fever | ||
| evidence: | ||
| - reference: PMID:8150635 | ||
| supports: SUPPORT | ||
| snippet: "This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia." | ||
| explanation: The abstract lists recurrent high fever as a typical symptom of Wissler-Fanconi syndrome. | ||
| - name: Polymorphous exanthema | ||
| category: Dermatological | ||
| frequency: FREQUENT | ||
| phenotype_term: | ||
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| preferred_term: Skin Rash | ||
| term: | ||
| id: HP:0000988 | ||
| label: Skin rash | ||
| evidence: | ||
| - reference: PMID:8150635 | ||
| supports: SUPPORT | ||
| snippet: "This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia." | ||
| explanation: The abstract lists polymorphous exanthemas as a typical symptom of Wissler-Fanconi syndrome. | ||
| - name: Arthralgia | ||
| category: Musculoskeletal | ||
| frequency: FREQUENT | ||
| phenotype_term: | ||
| preferred_term: Arthralgia | ||
| term: | ||
| id: HP:0002829 | ||
| label: Arthralgia | ||
| evidence: | ||
| - reference: PMID:8150635 | ||
| supports: SUPPORT | ||
| snippet: "This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia." | ||
| explanation: The abstract lists arthralgia as a typical symptom of Wissler-Fanconi syndrome. | ||
| - name: Leukocytosis | ||
| category: Hematologic | ||
| frequency: FREQUENT | ||
| phenotype_term: | ||
| preferred_term: Leukocytosis | ||
| term: | ||
| id: HP:0001974 | ||
| label: Increased total leukocyte count | ||
| evidence: | ||
| - reference: PMID:8150635 | ||
| supports: SUPPORT | ||
| snippet: "This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia." | ||
| explanation: The abstract lists leukocytosis as a typical symptom of Wissler-Fanconi syndrome. | ||
| - name: Microscopic hematuria | ||
| category: Renal | ||
| frequency: VERY_RARE | ||
| phenotype_term: | ||
| preferred_term: Microscopic hematuria | ||
| term: | ||
| id: HP:0002907 | ||
| label: Microscopic hematuria | ||
| evidence: | ||
| - reference: PMID:3572917 | ||
| supports: SUPPORT | ||
| snippet: "We describe a case of persistent microscopic hematuria as initial finding in incomplete Still's disease or Wissler-Fanconi syndrome." | ||
| explanation: The case report documents microscopic hematuria as a presenting sign. | ||
| - name: Arthritis | ||
| category: Musculoskeletal | ||
| frequency: FREQUENT | ||
| notes: Migratory polyarthritis reported in case presentations. | ||
| phenotype_term: | ||
| preferred_term: Arthritis | ||
| term: | ||
| id: HP:0001369 | ||
| label: Arthritis | ||
| evidence: | ||
| - reference: PMID:27843372 | ||
| supports: SUPPORT | ||
| snippet: "A middle-aged female presented to the emergency department with shortness of breath and chest pain associated with fever, polyarthritis, and had a chronic polymorphic rash on the back and lower extremities." | ||
| explanation: The case report documents polyarthritis as part of the clinical presentation. | ||
| - name: Pericarditis | ||
| category: Cardiovascular | ||
| frequency: VERY_RARE | ||
| phenotype_term: | ||
| preferred_term: Pericarditis | ||
| term: | ||
| id: HP:0001701 | ||
| label: Pericarditis | ||
| evidence: | ||
| - reference: PMID:3092775 | ||
| supports: SUPPORT | ||
| snippet: "The long-term evolution is marked by recurrent febrile exacerbations, sometimes complicated by pericardo-myocarditis which usually resolves without sequellae." | ||
| explanation: The abstract notes pericardo-myocarditis as a complication during the course of disease. | ||
| - name: Myocarditis | ||
| category: Cardiovascular | ||
| frequency: VERY_RARE | ||
| phenotype_term: | ||
| preferred_term: Myocarditis | ||
| term: | ||
| id: HP:0012819 | ||
| label: Myocarditis | ||
| evidence: | ||
| - reference: PMID:3092775 | ||
| supports: SUPPORT | ||
| snippet: "The long-term evolution is marked by recurrent febrile exacerbations, sometimes complicated by pericardo-myocarditis which usually resolves without sequellae." | ||
| explanation: The abstract reports pericardo-myocarditis as a complication of Wissler-Fanconi syndrome. | ||
| - name: Congestive heart failure | ||
| category: Cardiovascular | ||
| frequency: VERY_RARE | ||
| phenotype_term: | ||
| preferred_term: Congestive heart failure | ||
| term: | ||
| id: HP:0001635 | ||
| label: Congestive heart failure | ||
| evidence: | ||
| - reference: PMID:3092775 | ||
| supports: SUPPORT | ||
| snippet: "The authors report a case with chronic pericardo-myocarditis progressing over a 7 year period to refractory congestive cardiac failure." | ||
| explanation: The case report documents progression to refractory congestive heart failure. | ||
| biochemical: | ||
|
Contributor
There was a problem hiding this comment. The Biochemical class in the current schema does not have a
Options:
The existing entries are correctly capturing the presence/elevation status; adding formal term bindings would require schema modification. |
||
| - name: Erythrocyte sedimentation rate | ||
| presence: Elevated | ||
| evidence: | ||
| - reference: PMID:27843372 | ||
| supports: SUPPORT | ||
| snippet: "Blood sample analysis revealed high levels of CRP (42.9 mg/dL, normal: 0–0.3), erythrocyte sedimentation rate (113 mm/h normal: 2–20)" | ||
| explanation: The case report documents markedly elevated ESR, supporting a systemic inflammatory state. | ||
| - name: C-reactive protein | ||
| presence: Elevated | ||
| evidence: | ||
| - reference: PMID:27843372 | ||
| supports: SUPPORT | ||
| snippet: "Blood sample analysis revealed high levels of CRP (42.9 mg/dL, normal: 0–0.3), erythrocyte sedimentation rate (113 mm/h normal: 2–20)" | ||
| explanation: The case report documents markedly elevated CRP as part of the inflammatory syndrome. | ||
| progression: | ||
| - phase: Onset | ||
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| age_range: Childhood to adulthood | ||
| evidence: | ||
| - reference: PMID:8150635 | ||
| supports: SUPPORT | ||
| snippet: "A 20-year-old female patient with the typical signs of Wissler's subsepsis allergica (Wissler-Fanconi syndrome) is described." | ||
| explanation: This case report documents onset in a young adult. | ||
| - reference: PMID:1210460 | ||
| supports: SUPPORT | ||
| snippet: "A further case of subsepsis allergica Wissler in a 35-year-old woman is reported." | ||
| explanation: This report documents adult-onset disease. | ||
| - reference: DOI:10.1007/978-3-642-94912-8_6 | ||
| supports: SUPPORT | ||
| snippet: "Zunächst haben nur die Pädiater die Krankheit gesehen, neuerdings sind aber auch Fälle bei Erwachsenen bekanntgegeben worden." | ||
| explanation: The abstract notes that early reports were pediatric, with adult cases reported later. | ||
| - phase: Chronic course | ||
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| notes: Recurrent febrile exacerbations with possible cardiac complications. | ||
| evidence: | ||
| - reference: PMID:3092775 | ||
| supports: SUPPORT | ||
| snippet: "The long-term evolution is marked by recurrent febrile exacerbations, sometimes complicated by pericardo-myocarditis which usually resolves without sequellae." | ||
| explanation: The abstract summarizes recurrent febrile exacerbations and possible cardiac complications over time. | ||
| epidemiology: | ||
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| - name: Geographic distribution of reports | ||
| description: Early publications largely originated from continental Europe. | ||
| evidence: | ||
| - reference: DOI:10.1007/978-3-642-94912-8_6 | ||
| supports: SUPPORT | ||
| snippet: "Mit einer Ausnahme (N. Homma, Tokyo) stammen alle Publikationen von Kontinentaleuropa, die meisten aus Deutschland, Frankreich und Italien." | ||
| explanation: The abstract states most publications came from continental Europe, especially Germany, France, and Italy. | ||
| - name: Recognition in anglophone literature | ||
| description: The disease was not recognized in the English-speaking literature at the time of the review. | ||
| evidence: | ||
| - reference: DOI:10.1007/978-3-642-94912-8_6 | ||
| supports: SUPPORT | ||
| snippet: "Im angelsächsischen Sprachgebiet wird das Krankheitsbild bis jetzt nicht anerkannt." | ||
| explanation: The abstract notes lack of recognition in anglophone literature. | ||
| differential_diagnoses: | ||
| - name: Sepsis | ||
| description: Early Wissler-Fanconi presentations can be difficult to distinguish from septicemia. | ||
| distinguishing_features: | ||
| - Repeated blood and urine cultures can be negative despite high fever and leukocytosis. | ||
| disease_term: | ||
| preferred_term: infectious disease with sepsis | ||
| term: | ||
| id: MONDO:1040015 | ||
| label: infectious disease with sepsis | ||
| evidence: | ||
| - reference: PMID:8150635 | ||
| supports: SUPPORT | ||
| snippet: "In the early stages it is difficult to differentiate from septicaemia." | ||
| explanation: The abstract notes early overlap with septicemia. | ||
| - reference: PMID:27843372 | ||
| supports: SUPPORT | ||
| snippet: "Urine and multiple blood cultures were negative." | ||
| explanation: The case report documents negative cultures despite systemic inflammatory presentation. | ||
| - name: Acute rheumatic fever | ||
| description: Considered in the differential diagnosis because of overlapping febrile inflammatory presentations. | ||
| disease_term: | ||
| preferred_term: rheumatic fever | ||
| term: | ||
| id: MONDO:0017767 | ||
| label: rheumatic fever | ||
| evidence: | ||
| - reference: PMID:27843372 | ||
| supports: SUPPORT | ||
| snippet: "Features of Wissler-Fanconi syndrome can be found in a differential diagnosis that includes true sepsis, acute rheumatic fever, rheumatoid arthritis, and adult onset Still’s disease." | ||
| explanation: The case report explicitly lists acute rheumatic fever as part of the differential diagnosis. | ||
| - name: Rheumatoid arthritis | ||
| description: Inflammatory arthritis with systemic features can overlap with Wissler-Fanconi syndrome. | ||
| disease_term: | ||
| preferred_term: rheumatoid arthritis | ||
| term: | ||
| id: MONDO:0008383 | ||
| label: rheumatoid arthritis | ||
| evidence: | ||
| - reference: PMID:27843372 | ||
| supports: SUPPORT | ||
| snippet: "Features of Wissler-Fanconi syndrome can be found in a differential diagnosis that includes true sepsis, acute rheumatic fever, rheumatoid arthritis, and adult onset Still’s disease." | ||
| explanation: The case report explicitly lists rheumatoid arthritis as part of the differential diagnosis. | ||
| - name: Adult-onset Still's disease | ||
| description: Shares fever, rash, leukocytosis, and arthralgia; some authors consider Wissler-Fanconi syndrome related. | ||
| disease_term: | ||
| preferred_term: adult-onset Still disease | ||
| term: | ||
| id: MONDO:0019355 | ||
| label: adult-onset Still disease | ||
| evidence: | ||
| - reference: PMID:27843372 | ||
| supports: SUPPORT | ||
| snippet: "Features of Wissler-Fanconi syndrome can be found in a differential diagnosis that includes true sepsis, acute rheumatic fever, rheumatoid arthritis, and adult onset Still’s disease." | ||
| explanation: The case report explicitly lists adult-onset Still's disease as part of the differential diagnosis. | ||
| - reference: PMID:8150635 | ||
| supports: SUPPORT | ||
| snippet: "This syndrome has sometimes been considered equivalent to or an initial stage of Still's disease (juvenile rheumatoid arthritis) progressing to degenerative arthritis in many patients, whereas other authors have classified it as a separate entity with good prognosis." | ||
| explanation: The abstract notes debated overlap with Still's disease. | ||
| - name: Autoimmune polyendocrine syndrome type 1 | ||
| description: Reported in a case with subsepsis allergica; included as a differential context. | ||
| disease_term: | ||
| preferred_term: autoimmune polyendocrine syndrome type 1 | ||
| term: | ||
| id: MONDO:0009411 | ||
| label: autoimmune polyendocrine syndrome type 1 | ||
| evidence: | ||
| - reference: PMID:2233764 | ||
| supports: SUPPORT | ||
| snippet: "A 10.6 year old Turkish girl developed + the signs of a polyglandular autoimmune syndrome (PGA) type I since her first year of age." | ||
| explanation: The report describes a patient with type I polyglandular autoimmune syndrome alongside subsepsis allergica. | ||
| treatments: | ||
| - name: Nonsteroidal anti-inflammatory drugs | ||
| description: NSAIDs can reduce fever and inflammatory symptoms. | ||
| treatment_term: | ||
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| preferred_term: pharmacotherapy | ||
| term: | ||
| id: MAXO:0000058 | ||
| label: pharmacotherapy | ||
| qualifiers: | ||
| - predicate: | ||
| preferred_term: therapeutic agent | ||
| term: | ||
| id: NCIT:C2259 | ||
| label: Therapeutic Agent | ||
| value: | ||
| preferred_term: naproxen | ||
| term: | ||
| id: NCIT:C680 | ||
| label: Naproxen | ||
| evidence: | ||
| - reference: PMID:27843372 | ||
| supports: SUPPORT | ||
| snippet: "A combination of nonsteroidal anti-inflammatory drugs and steroids achieved complete remission." | ||
| explanation: The case report documents remission with NSAID therapy. | ||
| - name: Corticosteroids | ||
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| description: Systemic steroids are used to control inflammation. | ||
| treatment_term: | ||
| preferred_term: pharmacotherapy | ||
| term: | ||
| id: MAXO:0000058 | ||
| label: pharmacotherapy | ||
| qualifiers: | ||
| - predicate: | ||
| preferred_term: therapeutic agent | ||
| term: | ||
| id: NCIT:C2259 | ||
| label: Therapeutic Agent | ||
| value: | ||
| preferred_term: prednisolone | ||
| term: | ||
| id: NCIT:C769 | ||
| label: Prednisolone | ||
| evidence: | ||
| - reference: PMID:27843372 | ||
| supports: SUPPORT | ||
| snippet: "A combination of nonsteroidal anti-inflammatory drugs and steroids achieved complete remission." | ||
| explanation: The case report notes complete remission with steroids plus NSAIDs. | ||
| - reference: PMID:1210460 | ||
| supports: SUPPORT | ||
| snippet: "In the present case the disease could successfully be controlled using imuran in combination with corticosteroids." | ||
|
Contributor
There was a problem hiding this comment. Agreed - the evidence for dapsone clearly shows it was used as part of a 3-drug combination (naproxen + dapsone + prednisolone). Options:
The current schema may not have a formal "combination_therapy" slot. If this is a pattern seen across cancer entries, we could adopt that pattern here as well. |
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| explanation: The abstract indicates corticosteroids were part of successful therapy. | ||
| - name: Dapsone | ||
|
Member
There was a problem hiding this comment. I think this is fine but better to be explicit about combos |
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| description: Dapsone was used alongside NSAIDs and corticosteroids in a reported case. | ||
| treatment_term: | ||
| preferred_term: pharmacotherapy | ||
| term: | ||
| id: MAXO:0000058 | ||
| label: pharmacotherapy | ||
| qualifiers: | ||
| - predicate: | ||
| preferred_term: therapeutic agent | ||
| term: | ||
| id: NCIT:C2259 | ||
| label: Therapeutic Agent | ||
| value: | ||
| preferred_term: dapsone | ||
| term: | ||
| id: NCIT:C415 | ||
| label: Dapsone | ||
| evidence: | ||
| - reference: PMID:27843372 | ||
| supports: SUPPORT | ||
| snippet: "A combination of naproxen, dapsone, and prednisolone therapy resulted in significant improvement of the patient’s arthralgias and rash." | ||
| explanation: The case report documents improvement with dapsone as part of combination therapy. | ||
| - name: Azathioprine | ||
|
Contributor
There was a problem hiding this comment. Agreed - the azathioprine entry should clearly indicate combination therapy with corticosteroids. The evidence states "imuran in combination with corticosteroids." Suggested restructure: - name: Azathioprine plus corticosteroids
description: Immunosuppressive combination therapy using azathioprine with corticosteroids for disease control.
treatment_term:
preferred_term: immune suppressant agent therapy
term:
id: MAXO:0000297
label: immune suppressant agent therapy
notes: Combination regimen - azathioprine (imuran) with corticosteroidsOr if MAXO has a specific combination therapy term, that would be preferable. |
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| description: Immunosuppressive therapy used in combination with corticosteroids. | ||
| treatment_term: | ||
| preferred_term: pharmacotherapy | ||
| term: | ||
| id: MAXO:0000058 | ||
| label: pharmacotherapy | ||
| qualifiers: | ||
| - predicate: | ||
| preferred_term: therapeutic agent | ||
| term: | ||
| id: NCIT:C2259 | ||
| label: Therapeutic Agent | ||
| value: | ||
| preferred_term: azathioprine | ||
| term: | ||
| id: NCIT:C290 | ||
| label: Azathioprine | ||
| evidence: | ||
| - reference: PMID:1210460 | ||
| supports: SUPPORT | ||
| snippet: "In the present case the disease could successfully be controlled using imuran in combination with corticosteroids." | ||
| explanation: The abstract reports successful control with azathioprine (imuran) plus steroids. | ||
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| --- | ||
| reference_id: "DOI:10.1007/978-3-642-94912-8_6" | ||
| title: Subsepsis allergica | ||
| authors: | ||
| - Hans Wissler | ||
| journal: Ergebnisse der Inneren Medizin und Kinderheilkunde | ||
| year: '1965' | ||
| doi: 10.1007/978-3-642-94912-8_6 | ||
| content_type: abstract_only | ||
| --- | ||
|
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| # Subsepsis allergica | ||
| **Authors:** Hans Wissler | ||
| **Journal:** Ergebnisse der Inneren Medizin und Kinderheilkunde (1965) | ||
| **DOI:** [10.1007/978-3-642-94912-8_6](https://doi.org/10.1007/978-3-642-94912-8_6) | ||
|
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| ## Content | ||
| 1943 habe ich anhand von 5 Fällen unter dem Titel “Über eine besondere Form | ||
| sepsisähnlicher Krankheiten“ das Krankheitsbild der “Subsepsis hyperergica“ | ||
| beschrieben. Fanconi hat 1946 einen weiteren Fall mitgeteilt und den Namen in | ||
| „Subsepsis allergica“ umgeändert. Die Krankheit fand zunächst wenig Beachtung. | ||
| Einer ersten Arbeit von Keyzer in Holland 1946 folgten bis 1950 noch diejenige | ||
| von Künstler 1948 (jedoch unter dem Namen „Rheumatismus“) und von Fontan und | ||
| Verger 1948. Nach 1950 wurde das Krankheitsbild besser bekannt, so daß bis jetzt | ||
| 43 Publikationen zur Verfügung stehen. Dazu kommen noch einige mir im Original | ||
| nicht zugängliche aus Osteuropa, die bei Biedrzicka zitiert sind. 1958 und 1959 | ||
| habe ich das damals vorhandene eigene und fremde Material zusammengestellt (51, | ||
| 52). Mit einer Ausnahme (N. Homma, Tokyo) stammen alle Publikationen von | ||
| Kontinentaleuropa, die meisten aus Deutschland, Frankreich und Italien. Im | ||
| angelsächsischen Sprachgebiet wird das Krankheitsbild bis jetzt nicht anerkannt. | ||
| Sicher ist nur ein Teil der beobachteten Fälle veröffentlicht worden, wie mir | ||
| zahlreiche Anfragen gezeigt haben. — Zunächst haben nur die Pädiater die | ||
| Krankheit gesehen, neuerdings sind aber auch Fälle bei Erwachsenen | ||
| bekanntgegeben worden (23, 25, 38). |
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