Scleroderma (Progressive Systemic Sclerosis)


Epidemiology

Demographics

Risk Factors

Genetic Factors

Infection

Drugs

Environmental Exposures

Microchimerism


Physiology


Diagnosis

Erythrocyte Sedimentation Rate (ESR) (see Erythrocyte Sedimentation Rate)

Complete Blood Count (see Complete Blood Count)

CXCL4 Level

Serology

Antinuclear Antibody (ANA) (see Antinuclear Antibody)

Anticentromere Antibody (ACA) (see Anticentromere Antibody)

Antitopoisomerase-I Antibody (Anti-Scl-70) (see Antitopoisomerase-I Antibody)

Anti-PM/Scl Antibody

Anti-U1-RNP Antibody

Anti-U3 Nucleolar RNP Antibody

Rheumatoid Factor (RF) (see Rheumatoid Factor)

Circulating Immune Complexes (see xxxxx)

Ventilation-Perfusion (V/Q) Scan (see Ventilation-Perfusion Scan)


Clinical Criteria


Clinical Manifestations

Clinical Classification

Prescleroderma

Limited Cutaneous Scleroderma

Diffuse Cutaneous Scleroderma

Scleroderma Sine Scleroderma

Environmentally-Induced Scleroderma

Overlap Syndrome

Cardiovascular Manifestations

Capillary Changes of the Nail Beds

Digital Ulcers (see Mucocutaneous Ulcers)

Dry Skin

Lipoatrophy

Loss of Appendicular Hair

Pitting of the Fingertips

Pruritus (see Pruritus)

Sclerodactyly (see Sclerodactyly)

Skin Edema/Erythema

Skin Thickening/Hardening

Skin Hyperpigmentation/Depigmentation (“Salt and Pepper Skin”)

Telangiectasias (see Telangiectasias)

Vasculitis (see Vasculitis)

Gastrointestinal Manifestations

Hematologic Manifestations

Mild Thrombocytopenia (see Thrombocytopenia)

Neurologic Manifestations

Otolaryngologic Manifestations

Oropharyngeal Carcinoma (see Acute Respiratory Distress Syndrome)

Airway Disease

Aspiration Pneumonia (see Aspiration Pneumonia)

Diffuse Alveolar Hemorrhage (DAH) (see Diffuse Alveolar Hemorrhage)

Interstitial Lung Disease (ILD) (see Interstitial Lung Disease)

Lung Cancer (see Lung Cancer)

Pleural Effusion/Pleuritis (see Pleural Effusion-Exudate)

Pulmonary Hypertension (see Pulmonary Hypertension)

Secondary Pulmonary Hypertension Due to Scleroderma-Associated ILD (see Pulmonary Hypertension)

Pulmonary Veno-Occlusive Disease (VOD)/Pulmonary Capillary Hemangiomatosis (see Pulmonary Veno-Occlusive Disease and Pulmonary Capillary Hemangiomatosis)

Spontaneous Pneumothorax (see Pneumothorax)

Thoracic Cage Restriction (see Pulmonary Function Tests)

Venous Thromboembolism (see xxxx)

Renal/Genitourinary Manifestations

General Comments

Erectile Dysfunction (ED) (see Erectile Dysfunction)

Hypertension (see Hypertension)

Scleroderma Renal Crisis

Rheumatologic Manifestations


Prognosis


Treatment

Treatment of Esophageal Dysmotility with GERD Symptoms

Treatment of Interstitial Lung Disease

Clinical Efficacy

Treatment of Hypertension

Treatment of Raynaud Phenomenon (see Raynaud Phenomenon)

Treatment of Skin Disease


References

General

Diagnosis

Clinical

General

Cardiovascular Manifestations

Gastrointestinal Manifestations

Neurologic Manifestations

Pulmonary Manifestations

Treatment

Calcinosis Cutis

Lung Disease

Raynaud Phenomenon (see Raynaud Phenomenon)

Peripheral Arterial Disease

Prognosis