Intravenous Immunoglobulins May Be Effective in Certain Patients With Leucocytoclastic Vasculitis

Intravenous immunoglobulins (IVIG) may be an effective therapy for patients with leucocytoclastic vasculitis (LCV) following the failure of established systemic treatments, according to a study published in Sarcoidosis Vasculitis and Diffuse Lung Diseases. These results are based on a case report of a patient with LCV associated to sensitive neuropathy for whom systemic treatment was ineffective.

According to the investigators, when LCV is the manifestation of a systemic vasculitis process, treatment generally consists of a combination therapy comprised of steroids and immunosuppressive drugs. However, IVIG—when combined with traditional therapies—has been demonstrated to be effective in isolated severe cases with persistent ulcerations, suspected comorbid infection, or common variable immune deficiency.

In the case reviewed, the patient was unresponsive to a treatment of prednisone 25mg/day and dapsone 50mg/day, and continued to worsen when dapsone was replaced with azathioprine 100mg/day. During the patient’s hospitalization for worsening of their condition, they were given a single infusion of 2g/kg of IVIg distributed over 2 days. According to the investigators, this resulted in a dramatic improvement to the patient’s skin lesions, and the patient continued to demonstrate progressive clinical improvement after discharge when given 6 additional infusions of IVIG. These infusions were conducted once a month for 6 months and combined with prednisone 25 mg/day, gradually tapered, and azathioprine 100 mg/day.

“Three months after the last IVIg infusion, the patient was asymptomatic and neurological examination was negative, so oral prednisone was stopped but azathioprine 100 mg/day was maintained,” the researchers said in the study. “Actually, one year after the hospitalization, the patient continues the therapy with azathioprine 100 mg/day, but her clinical conditions are in stable remission, with occasional flare-ups on the skin, but no more signs of sensory neuropathy.”

Based on the results seen in the patient reviewed, the research team suggested that patients with extensive disease associated with neurologic involvement should begin IVIG treatment early in order to prevent permanent sequelae.

IVIG is generally associated with mild systemic adverse events, including headache, backache, myalgia, chills, fever, nausea or vomiting, and wheezing. Less commonly, patients may experience changes in blood pressure, tachycardia, aseptic meningitis, anaphylactic reactions, renal failure, and haemolytic anaemia.

REFERENCES

Ferranti M, Cama E, Cacciavillani M, et al. Leukocytoclastic vasculitis associated with multifocal sensory neuropathy responsive to intravenous immunoglobulins: a case report. Sarcoidosis Vasc Diffuse Lung Dis. 2021; 38(3): e2021022. doi:10.36141/svdld.v38i3.10883