Criteria for Rapidly (Fulminant) Progressive Systemic Sclerosis

Purpose: To determine if a patient with progressive systemic sclerosis (PSS) has rapidly progressive (fulminant) disease.

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Evaluation

Are you evaluating a patient with progressive systemic sclerosis?

Yes

Date of symptom onset (enter MM/DD/YY)

Date respiratory failure diagnosed (enter MM/DD/YY if failure, else leave blank)

Date renal failure diagnosed (enter MM/DD/YY if failure, else leave blank)

Date of heart failure diagnosed (enter MM/DD/YY if failure, else leave blank)

Results

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Results Interpretation and Description References Next Steps

Data complete?

Evaluation appropriate?

Have 3 years passed since symptom onset?

Number of organ failures within 3 years of symptoms onset

Does the patient meet the criteria for fulminant PSS?

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Interpretation and Description

References

Next Steps in Evaluation

Diagnosis

Risk Factors
- Scleroderma Risk Model for 5 Year Survival
Clinical Diagnosis, Including Family History For Genetics
- ARA Scleroderma Criteria Cooperative Study Preliminary Clinical Criteria for Systemic Sclerosis
Imaging Studies
Laboratory Tests
Other Testing
Criteria For Diagnosis
Severity, Prognosis, Stage
Differential Diagnosis And Mimics, Red Flags

Therapy

Physical And Lifestyle
- Drug Induced Scleroderma

Monitoring

Response To Therapy
Disease Progression
Comorbid Conditions
- Prognostic Factors for Patient with Juvenile Systemic Sclerosis
- Lung Severity Scale of Medsger for Patient with Systemic Sclerosis

Patient Concerns

End-of-life, Palliative Care
- Scleroderma Risk Model for 5 Year Survival

Specialty

Immunology/Rheumatology

Objective

laboratory tests
other testing
criteria for diagnosis
severity
prognosis
stage

ICD-10

M34 - Systemic sclerosis

Info

Purpose: To determine if a patient with progressive systemic sclerosis (PSS) has rapidly progressive (fulminant) disease.

Specialty: Immunology/Rheumatology

Objective: laboratory tests, other testing, criteria for diagnosis, severity, prognosis, stage

ICD-10: M34,

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