Systemic vasculitis may present with life-threatening complications that need to be promptly recognized and appropriately managed to ensure patient survival and minimize irreversible organ damage.
The most common life-threatening events differ between large and medium or small-vessel vasculitis. In large-vessel vasculitis, particularly giant-cell artertis (GCA), vascular remodelling in response to inflammation may lead to severe stenosis leading to ischemic stroke in 3–6% of patients and, rarely, myocardial infarction, mesenteric ischemia or critical extremity ischemia. For these complications, particularly when happening in treated patients, intensifying immunosuppressive therapy is not the best or only option and additional interventions may be required. Stroke occurs as a consequence of carotid or vertebral stenosis. Stenosis of the carotid siphon has been repeatedly reported. In necropsy studies, vasculitic involvement and thrombosis of proximal intracranial branches has been observed. Infarcts are usually multiple, usually happen shortly after the initiation of glucocorticoid therapy, convey a 30% mortality or lead to remarkable disability. If critical stenosis is suspected before irreversible infarction, percutaneous intraluminal angioplasty may be function and life saving.
Acute aortic syndrome (aortic dissection or intramural haematoma) is an increasingly recognized hurdle in patients with GCA. It is usually a delayed complication occurring months and frequently years after diagnosis. Its frequency has not been delimitated but in a recent prospective follow-up study it was demonstrated to affect at least 2% of patients. Emergency open surgery repair, when feasible, is the best option for ascending aorta involvement (type A) and endovascular repair for involvement of the descending aorta (type B).
In small-vessel vasculitis, life-threatening presentations include rapidly progressive glomerulonephritis leading to kidney failure, alveolar haemorrhage, alithiasic colecistitis with perforation and intestinal ischemia. Rapidly progressive glomerulonephritis and alveolar haemorrhage are more frequently seen in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and anti glomerular basement membrane disease. Alveolar haemorrhage can be occasionally seen in cryoglobulinemic or IgA vasculitis. In addition to supportive measures, these patients are usually treated with high-dose methyl-prednisolone, cyclophosphamide or rituximab and plasma exchange. Plasma exchange has been found superior to IV methyl prednisolone mega-doses in preserving or recovering renal function but this advantage do not seem to persist over long-term follow-up. This approach is usually applied also to alveolar haemorrhage although there is not strong evidence supporting it.
Gastrointestinal complications, particularly intestinal ischemia and intestinal or gallbladder perforation are life-threatening complications which may require emergent surgery. These may be observed in polyarteritis nodosa, cryoglobulinemic vasculitis, eosinophilic granulomatosis with polyangiitis and other AAV. High-dose glucocorticoids and cyclophosphamide are usually applied.Children with IgA vasculitis may develop bowel intussusception
Deep venous thrombosis and pulmonary embolisms are significantly more frequent in AAV and Behçet disease than in the general population, especially during active disease. Anti-coagulation may be needed in AAV, although this approach is controversial in Behçet's disease. By contrast, aneurysm formation is typical in polyarteritis nodosa and Behçet's disease and may be occasionally seen in AAV. Massive bleeding derived form aneurysm rupture usually requires arterial embolization.
It is important to keep in mind that during the early course of diagnosed vasculitis, intense immunosuppressive therapy may favour life-threatening infections including opportunistic infections such as pneumocystis jiroveci pneumonia or disseminated CMV.
In summary, systemic vasculitis may present with a variety of severe complications and other may develop during follow-up. These complications are heterogeneous, vary according to the size of vessels involved, and usually require specific procedures or treatments in addition to immunosuppressive therapy. Due to the life-threatening nature of these complications their immediate recognition and management are crucial to patient survival.
Supported by SAF 577–8R and Maratό TV3 2014/201507
Disclosure of Interest None declared
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