Therapy for Vasculitic Neuropathies

65-Year-Old Woman With Intermittent Fevers, Lower Extremity Paresthesia, Weight Loss, and Malaise

ANCA-associated renal vasculitis

Treatment of polyarteritis nodosa and Churg-Strauss syndrome: Indications of plasma exchanges

Churg-Strauss Angiitis

Clinicopathologic features of nonsystemic vasculitic neuropathy and microscopic polyangiitis-associated neuropathy: A comparative study

ObjectiveTo identify the most relevant antigens for monoclonal antibodies in lymphocytic infiltrates in non-systemic vasculitic neuropathy (NSVN).BackgroundCurrent immunosuppressive treatment for NSVN is insufficient. Monoclonal antibodies might be a treatment option,...

ATYPICAL VARIANT OF GRANULOMATOSIS WITH POLYANGIITIS WITH EOSINOPHILIA

Manifestations ophtalmologiques des vascularites systémiques : analyse de six observations et revue de la littérature

AB0582 NEUROLOGIC INVOLVEMENT IN ANTINEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED VASCULITIS –EXPERIENCE FROM A PORTUGUESE CENTER

The Neuropathies of Vasculitis.

Twenty-two patients with evidence of systemic vasculitis and peripheral neuropathy were clinically and electrophysiologically investigated in a retrospective study. Ten had a polyarteritis nodosa, 6 a probable polyarteritis nodosa, and 6 a Churg-Strauss syndrome. Nine patients presented clinically with mononeuropathy or mononeuropathy multiplex considered typical of ischemic involvement of peripheral nerve; nine had more diffuse neuropathy, two of them a symmetric polyneuropathy. EMG examination revealed more diffuse neuropathy than clinically predicted. Findings were of acute or subacute axonal neuropathy.

Rash and Pulmonary Infiltrates in a 37-Year-Old Woman

Immunoglobulin preparations for intravenous administration (IVIG) contain highly purified native molecules of human IgG, freed from high-molecular aggregates and vasoactive substances stabilized with glucose, maltose, glycine, sorbitol, etc. Intravenous immunoglobulins can be used as adjunctive therapy in systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APLS) with recurrent miscarriages. The aim of the clinical observation is to establish the efficacy of the treatment of immunovenin in a scheme, in parallel with pulse therapies with methylprednisolone (MP) and cyclophosphamide (CYC) in patients with active SLE, compared with the application of pulse therapies with methylprednisolone and cyclophosphamide alone. When comparing the effect of treatment with immunovenin 400 mg/kg body weight every 3 months for a period of 12 to 18 months, in parallel with pulse therapies with methylprednisolone and cyclophosphamide 1 g monthly intravenously in disease activity, a significant difference in positive influence on organic brain syndrome, cranial nerve function, arthritis/arthralgias and proteinuria. In both groups, the concentration of anti dsDNA and ACLA decreased to the same extent. Treatment with immunovenin has a positive effect on influencing disease activity in SLE, added to treatment with pulse therapies with methylprednisolone and cyclophosphamide.

Intravenous immunoglobulins in peripheral neuropathy associated with vasculitis

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 Behcet disease than in the general population, especially during active disease. Anti-coagulation may be needed in AAV, although this approach is controversial in Behcet9s disease. By contrast, aneurysm formation is typical in polyarteritis nodosa and Behcet9s 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