Scleroderma Questions & Answers

Updated: Sep 09, 2019
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Questions & Answers

Overview

What is scleroderma?

What is systemic sclerosis?

What are the types of systemic sclerosis?

What are the ACR/EULAR Revised Systemic Sclerosis Classification Criteria?

What is the pathophysiology of scleroderma?

What is the role of vascular alterations in the pathogenesis of scleroderma?

What causes scleroderma?

What is the prevalence of scleroderma in the US?

What is the global prevalence of scleroderma?

What are the racial predilections of scleroderma?

What are the sexual predilections of scleroderma?

Which age groups have the highest prevalence of scleroderma?

What is the prognosis of scleroderma?

What are the possible complications of scleroderma?

What are the mortality rates of scleroderma?

What is included in patient education about scleroderma?

Presentation

Which organ systems are affected by scleroderma?

What are the dermatologic signs and symptoms of scleroderma?

What are the signs and symptoms of vascular involvement in scleroderma?

What are the GI signs and symptoms of scleroderma?

What are the respiratory signs and symptoms of scleroderma?

What are the musculoskeletal signs and symptoms of scleroderma?

What are the cardiac signs and symptoms of scleroderma?

What are the renal signs and symptoms of scleroderma?

What are the genitourinary signs and symptoms of scleroderma?

What are the ocular and ENT signs and symptoms of scleroderma?

What are the neurologic and psychiatric signs and symptoms of scleroderma?

What are the constitutional signs and symptoms of scleroderma?

Which dermatologic findings are characteristic of scleroderma?

Which ENT findings are characteristic of scleroderma?

Which ocular findings are characteristic of scleroderma?

Which physical findings are characteristic of Raynaud phenomenon in scleroderma?

Which physical findings are characteristic of vascular involvement in scleroderma?

Which musculoskeletal findings are characteristic of scleroderma?

Which respiratory findings are characteristic of scleroderma?

Which GI findings are characteristic of scleroderma?

Which renal findings are characteristic of scleroderma?

Which cardiac findings are characteristic of scleroderma?

Which neurologic findings are characteristic of scleroderma?

Which genitourinary findings are characteristic of scleroderma?

DDX

Which conditions are included in the differential diagnoses of scleroderma?

Workup

How is scleroderma diagnosed?

Which lab tests are performed in the workup of scleroderma?

How is GI involvement assessed in scleroderma?

How is pulmonary involvement assessed in scleroderma?

How is cardiovascular involvement assessed in scleroderma?

What is the role of radiography in the workup of scleroderma?

How are microvascular alterations assessed in scleroderma?

What is the role of lab tests in the workup of scleroderma?

What is the role of autoantibody testing in the workup of scleroderma?

What is the role of CT scanning in the workup of scleroderma?

What is the role of pulmonary function tests in the workup of scleroderma?

What is the role of transthoracic echocardiography in the workup of scleroderma?

What is the role of ECG in the workup of scleroderma?

What is the role of endoscopy in the workup of scleroderma?

What is the role of nail-fold capillary microscopy in the workup of scleroderma?

What is the role of biopsy in the diagnosis of scleroderma?

Which pathologic changes are characteristic of scleroderma?

Which histologic findings are characteristic of scleroderma?

Treatment

How is scleroderma treated?

What is the role of HSCT in the treatment of scleroderma?

When is HSCT indicated for the treatment of scleroderma with pulmonary involvement?

What are pulmonary function test (PFT) criteria for pulmonary involvement in scleroderma?

What are the exclusion criteria for HSCT for the treatment of scleroderma with pulmonary involvement?

How is skin fibrosis in scleroderma treated?

How is pruritus treated in scleroderma?

How is Raynaud phenomenon treated in scleroderma?

How are digital ulcers treated in scleroderma?

How are the GI symptoms of scleroderma treated?

How is scleroderma pulmonary fibrosis treated?

How is pulmonary arterial hypertension (PAH) in scleroderma treated?

How is a scleroderma renal crisis treated?

How are the musculoskeletal symptoms of scleroderma treated?

How is scleroderma treated during pregnancy?

What is the role of surgery in the treatment of scleroderma?

Which dietary modifications are used in the treatment of scleroderma?

Which activity modifications are used in the treatment of scleroderma?

Which specialist consultations are beneficial to patients with scleroderma?

What is included in the long-term monitoring of scleroderma?

Medications

What is the role of medications in the treatment of scleroderma?

Which medications in the drug class PAH, PDE-5 Inhibitors are used in the treatment of Scleroderma?

Which medications in the drug class Endothelin receptor antagonist are used in the treatment of Scleroderma?

Which medications in the drug class Pulmonary, Tyrosine Kinase Inhibitors are used in the treatment of Scleroderma?

Which medications in the drug class Immunosuppressive agents are used in the treatment of Scleroderma?

Which medications in the drug class Corticosteroids are used in the treatment of Scleroderma?

Follow-up

What continued outpatient care is indicated in the treatment of scleroderma?

When is inpatient care indicated in the treatment of scleroderma?