Expert Perspectives On Clinical Challenges: Expert Perspectives: Challenges in Scleroderma

Arthritis Rheumatol. 2020 Sep;72(9):1415-1426. doi: 10.1002/art.41406. Epub 2020 Aug 9.

Abstract

You are consulted to evaluate a 56-year-old woman with known Raynaud's phenomenon, finger swelling of several; months' duration, and new hypertension with a blood pressure of 160/100 mm/Hg. She also reports progressive shortness of breath. Physical examination reveals telangiectasias, sclerodactyly, and proximal skin sclerosis (thick shiny skin on the chest and upper arms), and bibasilar crackles are found on chest examination. Laboratory tests reveal evidence of microangiopathic hemolytic anemia, thrombocytopenia, and elevation of the serum creatinine level (previously normal), and chest computed tomography shows evidence of ground-glass opacification in both lower lung fields.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adrenergic alpha-Antagonists / therapeutic use
  • Angiotensin Receptor Antagonists / therapeutic use
  • Angiotensin-Converting Enzyme Inhibitors / therapeutic use
  • Antibodies, Antinuclear / immunology
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Antihypertensive Agents / therapeutic use*
  • Antirheumatic Agents / therapeutic use*
  • Calcium Channel Blockers / therapeutic use
  • Complement C3 / immunology
  • Complement C4 / immunology
  • Complement Inactivating Agents / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Endothelin Receptor Antagonists / therapeutic use
  • Humans
  • Hypertension / diagnosis
  • Hypertension / etiology
  • Hypertension / therapy*
  • Indoles / therapeutic use
  • Lung Diseases, Interstitial / diagnostic imaging
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / therapy*
  • Plasma Exchange
  • Protein Kinase Inhibitors / therapeutic use*
  • RNA Polymerase III / immunology
  • Raynaud Disease
  • Renal Insufficiency / etiology
  • Renal Insufficiency / immunology
  • Renal Insufficiency / pathology
  • Renal Insufficiency / therapy*
  • Scleroderma, Diffuse / complications
  • Scleroderma, Diffuse / diagnosis
  • Scleroderma, Diffuse / immunology
  • Scleroderma, Diffuse / therapy*
  • Scleroderma, Limited / complications
  • Scleroderma, Limited / diagnosis
  • Scleroderma, Limited / immunology
  • Scleroderma, Limited / therapy*
  • Stem Cell Transplantation
  • Tomography, X-Ray Computed
  • Transplantation, Autologous
  • Vasodilator Agents / therapeutic use

Substances

  • Adrenergic alpha-Antagonists
  • Angiotensin Receptor Antagonists
  • Angiotensin-Converting Enzyme Inhibitors
  • Antibodies, Antinuclear
  • Antibodies, Monoclonal, Humanized
  • Antihypertensive Agents
  • Antirheumatic Agents
  • Calcium Channel Blockers
  • Complement C3
  • Complement C4
  • Complement Inactivating Agents
  • Endothelin Receptor Antagonists
  • Indoles
  • Protein Kinase Inhibitors
  • Vasodilator Agents
  • anticentromere antibody
  • Cyclophosphamide
  • eculizumab
  • RNA Polymerase III
  • nintedanib