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 


  1. Achkar AA, Stanson A W, Johnson C M et al (1995) Rheumatoid vasculitis manifesting as abdominal hemorrhage. Mayo Clin Proc 70(6):565–569Google Scholar
  2. Babian M, Nasef S, Soloway G (1998) Gastrointestinal infarction as a manifestation of rheumatoid vasculitis. Am J Gastroenterol 93:119–120PubMedCrossRefGoogle Scholar
  3. Bathon JM, Moreland LW, DiBartolomeo AG (1989) Inflammatory central nervous system involvement in rheumatoid arthritis. Semin Arthritis Rheum 18(4):258–266 PubMedCrossRefGoogle Scholar
  4. Breuer GS, Baer A, Dahan D et al (2006) Lupus-associated pancreatitis. Autoimmun Rev 5:314–318PubMedCrossRefGoogle Scholar
  5. Brillet PY, Brauner M (2007) Pulmonary imaging in ANCA-associated vasculitides. Presse Med 36(5 Pt 2):907–912PubMedCrossRefGoogle Scholar
  6. Burt RW, Berenson MM, Samuelson CO et al (1983) Rheumatoid vasculitis of the colon presenting as pancolitis. Dig Dis Sci 28:183–188PubMedCrossRefGoogle Scholar
  7. Caballol Pons N, Montalà N, Valverde J et al (2010) Isolated cerebral vasculitis associated with rheumatoid arthritis. Joint Bone Spine 77(4):361–363PubMedCrossRefGoogle Scholar
  8. Castañer E, Alguersuari A, Gallardo X et al (2010) When to suspect pulmonary vasculitis: radiologic and clinical clues. Radiographics 30:33–53PubMedCrossRefGoogle Scholar
  9. Chung SM, Lee CK, Lee EY et al (2006) Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension. Clin Rheumatol 25:866–872PubMedCrossRefGoogle Scholar
  10. Chung MP, Yi CA, Lee HY, Han J, Lee KS (2010) Imaging of pulmonary vasculitis. Radiology 255(2):322–341PubMedCrossRefGoogle Scholar
  11. Doyle MK (2006) Vasculitis associated with connective tissue disorders. Curr Rheumatol Rep 8:312–316PubMedCrossRefGoogle Scholar
  12. Drenkard C, Villa AR, Reyes E et al (1997) Vasculitis in systemic lupus erythematosus. Lupus 6:235–242PubMedCrossRefGoogle Scholar
  13. Ellis SG, Verity MA (1979) Central nervous system involvement in systemic lupus erythematosus: a review of neuropathologic findings in 57 cases, 1955–1977. Semin Arthritis Rheum 8:212–221PubMedCrossRefGoogle Scholar
  14. Frankel SK, Cosgrove GP, Fischer A et al (2006) Update in the diagnosis and management of pulmonary vasculitis. Chest 129:452–465PubMedCrossRefGoogle Scholar
  15. Geboes K, Dalle I (2002) Vasculitis and the gastrointestinal tract. Acta Gastroenterol Belg 65:204–212PubMedGoogle Scholar
  16. Genta MS, Genta RM, Gabay C (2006) Systemic rheumatoid vasculitis: a review. Semin Arthritis Rheum 36(2):88–98PubMedCrossRefGoogle Scholar
  17. Graham JW, Jan W (2003) MRI and the brain in systemic lupus erythematosus. Lupus 12:891–896PubMedCrossRefGoogle Scholar
  18. Guillevin L (2009) Vasculopathy and pulmonary arterial hypertension. Rheumatology (Oxford) 48(Suppl 3):iii54–iii57CrossRefGoogle Scholar
  19. Ha HK, Lee SH, Rha SE et al (2000) Radiologic features of vasculitis involving the gastrointestinal tract. Radiographics 20:779–794PubMedGoogle Scholar
  20. Haas C (2004) Pulmonary hypertension associated with systemic lupus erythematosus. Bull Acad Natl Med 188:985–997PubMedGoogle Scholar
  21. Hahn BH (2003) Systemic lupus erythematosus and accelerated atherosclerosis. N Engl J Med 349(25):2379–2380PubMedCrossRefGoogle Scholar
  22. Hajj-Alia RA, Calabrese LH (2009) Central nervous system vasculitis. Current Opinin Rheumatol 21:10–18CrossRefGoogle Scholar
  23. Hanly JG, Walsh NM, Sangalang V (1992) Brain pathology in systemic lupus erythematosus. J Rheumatol 19:732–741PubMedGoogle Scholar
  24. Helliwell TR, Flook D, whitworth J et al (1985) Arteritis and venulitis in systemic lupus erythematosus resulting in massive lower intestinal haemorrhage. Histopathology 9:1103–1113PubMedCrossRefGoogle Scholar
  25. Iyngkaran P, Limaye V, Hill C et al (2003) Rheumatoid vasculitis following influenzae vaccination. Rheumatology 42:907–909PubMedCrossRefGoogle Scholar
  26. Ju JH, Min JK, Jung CK et al (2009) Lupus mesenteric vasculitis can cause acute abdominal pain in patients with SLE. Nat Rev Rheumatol 5:273–281PubMedCrossRefGoogle Scholar
  27. Kalunian K, Joan TM (2009) New directions in the treatment of systemic lupus erythematosus. Curr Med Res Opin 25(6):1501–1514 PubMedCrossRefGoogle Scholar
  28. Kitas G, Banks MJ, Bacon PA (2001) Cardiac involvement in rheumatoid disease. Clin Med 1(1):18–21PubMedGoogle Scholar
  29. Knockaert DC (2007) Cardiac involvement in systemic inflammatory diseases. Eur Heart J 28(15):1797–1804PubMedCrossRefGoogle Scholar
  30. Ko SF, Lee TY, Chang TT et al (1997) Findings at lupus mesenteric vasculitis. Acta Radiol 38:115–120PubMedGoogle Scholar
  31. Kurata A, Kawakami T, Sato J et al (2010) Aortic aneurysms in systemic lupus erythematosus a meta-analysis of 35 cases in the literature and two different pathogeneses. Cardiovasc Pathol 20(1):e1–e7PubMedCrossRefGoogle Scholar
  32. Kurne A, Karabudak R, Karadag O et al (2009) An unusual central nervous system involvement in rheumatoid arthritis: combination of pachymeningitis and cerebral vasculitis. Rheumatol Int 29(11):1349–1353PubMedCrossRefGoogle Scholar
  33. Nakamura H, Okada A, Kawakami A et al (2010) Rheumatoid vasculitis of crural muscles confirmed by muscle biopsy in the absence of inflammatory myopathy: histologic and MRI study. Rheumatol Int 30(10):1381–1383PubMedCrossRefGoogle Scholar
  34. Nobrega TP, Klodas E, Breen JF et al (1996) Giant coronary artery aneurysms and myocardial infarction in a patient with systemic lupus erythematosus. Cathet Cadiovasc Diagn 39:75–79CrossRefGoogle Scholar
  35. Passam FH, Diamantis ID, Perisinaki G et al (2004) Intestinal ischemia as the first manifestation of vasculitis. Semin Arthritis Rheum 34(1):431–441PubMedCrossRefGoogle Scholar
  36. Ramos-Casals M, Nardi N, Lagrutta et al (2006) Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients. Medicine 85:95–104Google Scholar
  37. Renaudineau Y, Dugue C, Dueymes M et al (2002) Antiendothelial cell antibodies in systemic lupus erythematosus. Autoimmun Rev 1:365–372PubMedCrossRefGoogle Scholar
  38. Sanchez O, Sitbon O, Jaı¨s X et al (2006) Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 130:182–189PubMedCrossRefGoogle Scholar
  39. Scott DG, Bacon PA, Tribe CR (1981) Systemic rheumatoid vasculitis: a clinical and laboratory study of 50 cases. Medicine 60:288–297PubMedCrossRefGoogle Scholar
  40. Sharma AK, Farb A, Maniar P et al (2003) Spontaneous coronary artery dissection in a patient with systemic lupus erythematosis. Hawaii Med J 62:248–253PubMedGoogle Scholar
  41. Struthers GR, Scott DL, Delamere JP et al (1981) Smoking and rheumatoid vasculitis. Rheumatol Int 1:145–146PubMedCrossRefGoogle Scholar
  42. Suzuki H, Fujigaki Y, Mori M (2009) Giant coronary aneurysm in a patient with systemic lupus erythematosus. Intern Med 48:1407–1412PubMedCrossRefGoogle Scholar
  43. Tian XP, Zhang X (2010) Gastro-intestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment. World J Gastroenterol 28; 16(24):2971–2977Google Scholar
  44. Tsokos GC, Tsokos M, Le Riche NGH et al (1986) A clinical and pathologic study of cerebrovascular disease in patients with systemic lupus erythematosus. Sem Arthritis Rheumatism 16:(1):70–78Google Scholar
  45. Turesson C, Jacobsson LT (2004) Epidemiology of extra-articular manifestations in rheumatoid arthritis. Scand J Rheumatol 33:65–72PubMedCrossRefGoogle Scholar
  46. Turesson C, Schaid DJ, Weyand CM et al (2005) The impact of HLA-DRB1 genes on extra-articular disease manifestations in rheumatoid arthritis. Arthritis Res Ther 7:1386–1393CrossRefGoogle Scholar
  47. Turesson C, Schaid DJ, Weyand CM et al (2006) Association of smoking and HLAC3 with vasculitis in patients with rheumatoid arthritis. Arthritis Rheum 54:2276–2283Google Scholar
  48. Turessona C, Matteson EL (2009) Vasculitis in rheumatoid arthritis. Current Opin Rheumatol 21:35–40CrossRefGoogle Scholar
  49. Van Laar JM, Smit VT, de Beus WM et al (1998) Rheumatoid vasculitis presenting as appendicitis. Clin Exp Rheumatol 16:736–738PubMedGoogle Scholar
  50. Voskuyl AE (2006) The heart and cardiovascular manifestations in rheumatoid arthritis. Rheumatology 45(Suppl 4):iv4–iv7PubMedCrossRefGoogle Scholar
  51. Watts RA, Mooney J, Lane SE et al (2004) Rheumatoid vasculitis: becoming extinct? Rheumatology 43(7):920–923PubMedCrossRefGoogle Scholar
  52. Weyand CM, Xie C, Goronzy JJ (1992) Homozygosity for the HLA-DRB1 allele selects for extraarticular manifestations in rheumatoid arthritis. J Clin Invest 89:2033–2039PubMedCrossRefGoogle Scholar
  53. Zamora MR, Warner ML, Tuder R et al (1997) Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome. Medicine 76(3):192–202PubMedCrossRefGoogle Scholar

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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