Vasculitis Associated with Connective Tissue Disease

  • Nadia Mama-Larbi
  • Kalthoum Tlili-Graiess
  • Anis Askri
  • Ahmed Laatar
  • Amira Manamani
  • Leith Zakraoui
  • Lotfi Hendaoui
Part of the Medical Radiology book series (MEDRAD)


Vasculitis secondary to connective tissue disorders most commonly arises in the context of preexisting rheumatoid arthritis, systemic lupus erythematosus, or primary Sjögren’s syndrome. In patients with established connective tissue disorders, vasculitis may involve vessels of any size, but small vessel involvement predominates. The presence of vasculitis is usually correlated with disease activity. The clinical presentation ranges from isolated cutaneous involvement to life-threatening internal organ involvement. Tissue biopsy is the gold standard for diagnosis. Vasculitis secondary to systemic lupus erythematosus is common (11–50%). Cutaneous involvement is the most frequent form. Pulmonary involvement is manifested by diffuse alveolar hemorrhage. Chest radiographs shows alveolar infiltrates and CT shows diffuse ill-defined centrilobular nodules or small ground-glass centrilobular opacities. MRI in neurological involvement shows white matter lesions that are not specific and brain infarction. Other organ involvements that can be diagnosed by imaging are uncommon: gastrointestinal tract, coronary arteries, and the aorta. Vasculitis secondary to rheumatoid arthritis is common; autopsy data have reported systemic vasculitis ranging from 15 to 31%. Cardiac involvement includes pericarditis and coronary vasculitis. MRI in neurological vasculitis shows leptomeningitis and parenchymal ischemia. Abdominal vasculitis including ischemic bowel, intra-abdominal hemorrhage, and pancreatitis is rare. Imaging is not specific of vasculitis in these cases. Pulmonary artery involvement is uncommon.


Systemic Lupus Erythematosus Systemic Lupus Erythematosus Patient Systemic Vasculitis Pulmonary Artery Hypertension Diffuse Alveolar Hemorrhage 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer-Verlag Berlin Heidelberg  2011

Authors and Affiliations

  • Nadia Mama-Larbi
    • 1
  • Kalthoum Tlili-Graiess
    • 1
  • Anis Askri
    • 2
  • Ahmed Laatar
    • 3
  • Amira Manamani
    • 2
  • Leith Zakraoui
    • 3
  • Lotfi Hendaoui
    • 2
  1. 1.Department of Medical Imaging, Medical School of SousseSahloul University HospitalSousseTunisia
  2. 2.Department of Medical Imaging and Imaging Guided Interventions, Medical School of TunisMongi Slim University HospitalLa Marsa, Sidi DaoudTunisia
  3. 3.Department of Rheumatology, Medical School of TunisMongi Slim University HospitalLa Marsa, Sidi DaoudTunisia

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