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In medicine, vasculitis (plural: vasculitides) is a group of diseases featuring inflammation of the wall of blood vessels. Its main causes are autoimmune disorders and (occasionally) infections. Treatment depends on the cause. While most vasculitides are rare diseases, they generally affect several organ systems and can cause severe disability. more...

VACTERL association
Van der Woude syndrome
Van Goethem syndrome
Varicella Zoster
Variegate porphyria
Vasovagal syncope
VATER association
Velocardiofacial syndrome
Ventricular septal defect
Viral hemorrhagic fever
Vitamin B12 Deficiency
VLCAD deficiency
Von Gierke disease
Von Hippel-Lindau disease
Von Recklinghausen disease
Von Willebrand disease


The types of vasculitis are distinguished by the type of blood vessel affected (aorta, large arteries, arterioles, capillaries and veins), the appearance of biopsy tissue of affected organs on light microscopy, and if necessary, with the help of immunohistochemistry (use of monoclonal antibodies against specific inflammatory protein markers).

Other diagnostic tools are the detection of circulating antibodies that are associated with forms of vasculitis. While these measurements have a low positive and negative predictive value (due to the high rates of both false positives and false negatives), they can direct the clinician to specific causes for vasculitis.


Infectious vasculitis is generally treated with directed antibiotics, while autoimmune forms often require treatment with immune suppression: steroids, DMARDs ("steroid-sparing agents") or cyclophosphamide (a mild form of chemotherapy). For very severe forms, bone marrow transplantation is presently being investigated as the ultimate silencing of the immune system.

Causes and types

  • Large vessel vasculitis
    • Giant cell arteritis (also temporal arteritis)
    • Takayasu's arteritis
  • Medium-sized vessel vasculitis
    • Polyarteritis nodosa
    • Kawasaki's disease
    • Cerebral vasculitis (primary granulomatous)
  • Small-vessel vasculitis
    • Associated with ANCAs (anti-neutrophil cytoplasmatic antibody):
      • Microscopic polyangiitis
      • Wegener's granulomatosis
      • Churg-Strauss syndrome
      • Drug-induced
    • Associated with deposition of immune complexes:
      • Henoch-Schönlein purpura (HSP)
      • Cryoglobinemic vasculitis
      • Lupus erythematosus vasculitis
      • Rheumatoid vasculitis
      • Sjögren's syndrome vasculitis
      • Urticarial vasculitis associated with decreased complement
      • Behçet's disease
      • Goodpasture's syndrome
      • Serum sickness-vasculitis
      • Drug-induced
      • Infection-induced (not infectious)
    • Paraneoplastic
      • Lympho- and myeloproliferative neoplasm associated
      • Carcinoma-associated
    • Inflammatory bowel disease vasculitis


  • Jenette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997; 337(21):1512-23. PMID 9366584.


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C-Antineutrophil cytoplasmic antibody vasculitis in a patient with primary pulmonary hypertension on prostacyclin and bosentan
From CHEST, 10/1/05 by Aneesa M. Das

INTRODUCTION: Pulmonary arterial hypertension (PAH) and Wegener's granulomatosis are both rare diseases. To our knowledge these have not been described in the same patient. Although there is an association of skin manifestations with epoprostenol and bosanten, there are no known associations of C-antineutrophil cytoplasmic antibody (ANCA) vasculitis with the use of these drags.

CASE PRESENTATION: A thirty-nine year old woman, previously diagnosed with PAH, presented with a two day history of a non-pruritic, non-painful rash over her lower extremities. She denied any fevers, flushing episodes, hematuria, presyncope, or the use of new medications or new skin products. Her past medical history included seizures and tracheotomy for bilateral ankylosed arytenoids identified after an intubation three years prior. Her medications included prostacyclin, bosentan, warfarin, and depakote. She had been seen 3 weeks prior to presentation to increase her epoprostenol from 12 to 13 ng/kg/min. On physical exam, she had a non-blanching purple maculopapular rash over her lower extremities bilaterally with some confluent areas. She had minimal peripheral edema. She had a normal pulmonary exam and a pronounced P2 on cardiovascular exam. A biopsy of the rash confirmed leukocytoclastic vasculitis (LCV) (Figure 1). Her serologic work up included the following: normal liver enzymes, erythrocyte sedimentation rate 87 mm/ hr, anti-nuclear antibody 1:80, platelets 113 M/mL, negative myeloperoxidase-ANCA, positive proteinase 3 (PR 3) ANCA at 173.1 u/mL. Her creatinine was 0.9 mg/dL and she had 2+ protein and 4+ blood in her urine with dysmorphic red cells present. A subsequent renal biopsy showed focal necrotizing pauci-immune, PR3-ANCA associated glomerulonephritis. She subsequently began treatment with intravenous cyclophosphamide and prednisone. After one month of this therapy, she presented with hemoptysis. Her computed tomography scan (Figure 2) was consistent with pulmonary alveolar hemorrhage. She received plasmapheresis and pulse steroids. Her hemorrhage stabilized and she was discharged. Unfortunately, the patient's PAH has been too unstable to wean or discontinue the epoprostenol or bosentan. She continues to have dysmorphic red cells on urinalysis despite treatment.


DISCUSSIONS: This woman could have developed two distinct life threatening diseases or relationships could potentially exist between this patient's ANCA vasculitis, her PAH and its treatment. First, the patient could have had an initial smoldering vasculitis causing endothelial damage and predisposing the patient to PAH. Although the patient was intubated for less than 2 weeks in 2001, she underwent tracheotomy for ankylosed arytenoids which could have been associated with Wegener's granulomatosis. Secondly, the vasculitis could have been induceaby the epoprostenol. LCV has been previously reported to be associated with epoprostenol, however system vasculitis was not reported (1). PR3-ANCA has an effect on prostacyclin synthesis by endothelial cells in vitro, therefore suggesting a possible relationship between the two (2). Notably, her ANCA titer was negligible prior to initiation of epoprostenol. Finally, the vasculitis could be induced by bosentan. Bosentan has also been reported to be associated with necrotizing LCVa. Our patient had been on a stable dose of 125mg twice daily throughout the entire course of her vasculitis; however her rash intensity and ANCA levels varied throughout.

CONCLUSION: In summary, we describe a patient who developed Wegener's granulomatosis while undergoing treatment with epoprostenol and bosanten for her PAH. Although a direct causal relationship can not be asserted, an association between epoprostenol or bosentan and ANCA vasculitis is possible.


(1) Myers SA et al. Cutaneous findings in patients with pulmonary arterial hypertension receiving long-term epoprostenol therapy. J Am Acad Dermatol 2004;51(1):98-102

(2) Sibelius U et al. Wegener's granulomatosis: anti-proteinase 3 antibodies are potent inductors of human endothelial cell signaling and leakage response. J Exp Med 1998;187(4):497-503

(3) Stefan Get al. Severe necrotizing leucocytoclastic vasculitis in a patient taking bosentan. BMJ 2004;329:430

DISCLOSURE: Aneesa Das, None.

Aneesa M. Das MD * Linda Paradowski MD University of North Carolina Hospitals, Durham, NC

COPYRIGHT 2005 American College of Chest Physicians
COPYRIGHT 2005 Gale Group

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