- Author(s): Pascal Pillet
, Hôpital Pellegrin-Enfants, CHU, Département de pédiatrie, Place Amélie Raba-Léon, 33076 Bordeaux, France
- Key words: Takayasu, antineutrophil cytoplasm antibodies, Wegener, microscopic polyangiitis, Churg and Strauss
- Page(s) : 377-86
- DOI : 10.1684/mtp.2011.0408
- Published in: 2011
The childhood anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (i.e. Churg-Strauss syndrome, Wegener's granulomatosis and microscopic polyangiitis) & Takayasu disease are rare and severe. The cause is unknown and treatment is largely on experience gained from treating adult patients. TA is a rare chronic relapsing large vessel vasculitis affecting the aorta and its major branches and pulmonary arteries. Diagnosis of this exceptionally rare childhood vasculitis whose clinical manifestations are varied is tricky and often delayed as no TA specific biologic markers has been identified. ANCA-associated vasculitides predominantly involve small to medium-sized arteries. Pulmonary & renal involvement are common to these similar entities. Much progress has been gained on knowledge and care of these patients, standard of care is based on the use of immunosuppressants and in the most severe cases on a combination of glucocorticoids and cyclophosphamide.