Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a group of potentially life-threatening autoimmune diseases. A recent development in this field is the recognition that certain drugs can induce AAV. Among these agents, the drug most often implicated in causing disease is the commonly used antithyroid agent propylthiouracil (PTU). This Review provides an update on PTU-induced AAV. Clinical characteristics of PTU-induced AAV are similar to that of primary AAV, but usually have a milder course and better prognosis, provided early cessation of the disease-causing drug. PTU-induced ANCAs usually react to several components of myeloid granules, which is helpful in differentiating PTU-induced AAV from primary AAV. Early cessation of PTU is crucial in the treatment of PTU-induced AAV. The duration of immunosuppressive therapy might be shorter than in primary AAV, depending on the severity of organ damage, and maintenance therapy is not always necessary.

Propylthiouracil-induced antineutrophil cytoplasmic antibody-associated vasculitis

The vasculopathic reaction pattern

Propylthiouracil is a common medication used in patients with hyperthyroidism; it can cause perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) in some patients with Graves' disease. This antibody has been associated with various forms of vasculitis and neutrophilic dermatosis. Herein, we report a patient who presented with cutaneous manifestations of pyoderma gangrenosum with simultaneous development of p-ANCAs during PTU therapy for Graves' disease.

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A Case of p-ANCA-Positive Propylthiouracil-Induced Pyoderma Gangrenosum

Background Propylthiouracil (PTU), one of the mainstays of antithyroid therapy drugs, can lead to antineutrophil cytoplasmic antibody (ANCA) positivity and skin lesions. PTU-induced ANCA-positive vasculitis is rare and even more rare is erythema nodosum.



Objective  To report a case of a 57-year-old woman with hyperthyroidism who developed myeloperoxidase (MPO)-ANCA erythema nodosum after PTU treatment for 11 months.



Methods  Skin biopsy demonstrated septal panniculitis without vasculitis. PTU-induced ANCA-positive erythema nodosum was made.



Results  With discontinuation of PTU and initiation of thalidomide, skin lesions resolved completely in three weeks, and after three months, the titers of MPO-ANCA and perinuclear-ANCA (p-ANCA) had decreased remarkably. At 14-month follow-up, the patient was asymptomatic, but low levels of ANCA titers persisted.



Conclusions  This report indicated that ANCA positive erythema nodosum could develop following PTU treatment. Thalidomide has been proven to be helpful and averted the adverse effects from systemic corticosteroids and other immunosuppressive drugs in this patient.

Propylthiouracil-induced ANCA-positive erythema nodosum treated with thalidomide.

The purpose of this case report and review of the literature is to provide an exploration of the clinical symptoms, diagnosis, prevention, and management of propylthiouracil (PTU)-associated vasculitis in the intensive care setting. A PubMed search of the available literature was conducted using the MeSH search terms "propylthiouracil" and "vasculitis." The literature search returned 121 articles. Twenty-five were excluded because they were not in English. Fifty-nine case reports or case studies describing PTU-associated vasculitis were included. Data extracted from each case study included patient age, sex, autoimmune markers, laboratory tests, length of time on PTU, treatment for vasculitis, and patient outcomes. The authors reviewed 128 cases of PTU-associated vasculitis. The majority were women (8.8:1 F:M ratio), and the most common presenting symptoms were rash (51.6%), fever (46.9%), and arthralgia (43.8%). In addition to discontinuing PTU, the most common treatment was steroids (71.9%). Eight patients (6.3%) progressed to end-stage renal disease; two (1.6%) required intubation for respiratory failure; and five (3.9%) died of various organ systems failure related to vasculitis development. A high index of suspicion for vasculitis should be maintained, especially when presented with skin manifestations in the presence of PTU therapy. Screening with myeloperoxidase-antinuclear cytoplasmic antibodies is most sensitive. Positive screening should prompt a thorough clinical investigation. In cases of severe skin manifestations, the focus should be on aggressive wound care. Our case report is unique, not only in the size and extent of cutaneous involvement, but also as the first description of mortality secondary to cutaneous manifestations.

Propylthiouracil-Associated Leukocytoclastic Necrotizing Cutaneous Vasculitis: A Case Report and Review of the Literature.

Administration of propylthiouracil therapy has been associated with a hypersensitivity syndrome that typically manifests as vasculitis. 1 Most cases of propylthiouracil therapy-induced antineutrophil cytoplasmic antibody (ANCA) positivity react to perinuclear ANCA (p-ANCA). 1 We describe a patient who presented with cutaneous manifestations of propylthiouracil therapy hypersensitivity vasculitis with ANCA positivity.

https://jamanetwork.com/journals/jamadermatology/articlepdf/406375/dob60007.pdf

Hsin-Su Yu

Papers

Antineutrophil cytoplasmic antibody-positive cutaneous leukocytoclastic vasculitis associated with propylthiouracil therapy.