Management of Primary Small-Vessel Vasculitis

Crystal E. Nwannunu, BS; Radhika Shah, BS, MS; Allison L. Limmer, BS, BA; Ravi R. Patel, MD; Uyen Ngoc Mui, MD; Stephen K. Tyring, MD


Skin Therapy Letter. 2020;25(3):5-8. 

In This Article

Current Management

Management of SVV is based on the severity of systemic involvement, skin lesions, and treatment of any underlying comorbidities. A multidisciplinary approach involving rheumatology, pulmonology, nephrology, and others is often beneficial in severe cases. The most common and effective therapies for SVV can be found in Table 1.

Of note, while the majority of IgAV cases require symptomatic treatment only (i.e., managing arthropathy and abdominal pain with rest and analgesia), preventative measures are attempted to manage associated renal disease.[18] Although there are multiple therapeutic agents used for renal disease intervention, their treatment efficacy is still being debated. A meta-analysis of 13 randomized controlled trials was conducted to analyze the benefits and harms of these agents compared to placebo in the prevention and treatment of kidney disease in adults and children. Results revealed no evidence of benefit in the use of prednisone or antiplatelet agents in preventing kidney disease in children with IgAV, and no evidence of benefit has been found for cyclophosphamide treatment in adults or children with severe kidney disease.[23]

Management of cutaneous lesions consists of providing supportive care, avoiding triggers, assessing skin lesion severity, and treating the underlying systemic disease. For mild and non-ulcerative skin lesions, supportive measures including leg elevation, gradient support hose, and avoidance of tight clothing, sun exposure, and cold temperatures are recommended. Medications such as antihistamines, topical steroids and topical calcineurin inhibitors can be helpful to alleviate skin symptoms. Antibiotics should also be employed when there is an associated infection. High-dose steroids can be used to treat patients with symptoms of ulcerative cutaneous lesions and signs of minimal systemic disease. It is recommended that high-dose prednisone of up to 1 mg/kg/day be given along with a slow 4–6 week taper to limit some of the severe side effects of long-term systemic corticosteroid use. If recurrent vasculitis occurs during tapering, the addition of a steroid-sparing agent may reduce a patient's exposure to high-dose steroid therapy. Helpful agents include methotrexate (MTX) at <25 mg weekly after proper evaluation of the patient's creatinine clearance or azathioprine at 2 mg/kg/day. For patients displaying a more severe cutaneous/systemic presentation, pulse doses of prednisone can be given intermittently instead of a long taper.[2]

Lastly, since comorbid conditions such as hypertension, diabetes, hypercholesterolemia, and smoking can accelerate vascular damage, appropriate management of these diseases and cessation of smoking should be highly recommended.[1]