Low-Dose Methotrexate Controls a Severe Form of Polyarteritis Nodosa

Boehm, Ingrid; Bauer, R.

doi:10.1001/archderm.136.2.167

Cited by 20 publications

(19 citation statements)

References 9 publications

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“…Remissions have been reported, but upon tapering the steroid dose exacerbations of CPAN are generally reported [3]. Steroid sparing therapies reported for CPAN include hydroxychloroquine, dapsone, azathioprine, methotrexate, IvIg, and cyclophosphamide [10][11][12][13]. Although immunosuppressives have been reported to be effective in CPAN, there are no controlled prospective trials to evaluate their efficacy.…”

Section: Discussionmentioning

confidence: 99%

Refractory cutaneous polyarteritis nodosa with threat of loss of feet: successful treatment with rituximab

Stephan¹,

Minna²

2017

J Rheumatol Orthop

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Cutaneous Polyarteritis Nodosa (CPAN) is a rare, single organ vasculitis of unknown etiology which presents with livedo reticularis and tender nodules involving the lower extremity in 97% of cases. Diagnosis is established by skin biopsy demonstrating segmental leukocytoclastic vasculitis and fibrinoid necrosis with workup excluding any systemic involvement ( Figure 1). The presence of IgM antiphosphatidylserine-prothrombin complexes has led to the hypothesis that CPAN is a localized arthrus reaction. CPAN is characterized as a benign, chronic, relapsing vasculitis to be treated conservatively. As a single organ vasculitis, CPAN can affect arteries of any size. Due to CPAN's predilection for the lower extremities, involvement of the limb at or below the ankle's major arteries may result in loss of digits and potentially feet. The case presented involves CPAN in which the main arteries to the feet are occluded. The patient had been refractory to therapy with findings of early digital necrosis. Following adequate skin biopsy to establish a diagnosis, we initiated targeted therapy with rituximab, azathioprine, and high dose steroids with reversal of the ischemic process. It is the authors' opinion that CPAN should be treated more aggressively from initial diagnosis to prevent morbidity and potentially abort a chronic relapsing course.

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Section: Discussionmentioning

confidence: 99%

Refractory cutaneous polyarteritis nodosa with threat of loss of feet: successful treatment with rituximab

Stephan¹,

Minna²

2017

J Rheumatol Orthop

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“…Given alone or in association with corticosteroids, the benefit of MTX has been reported in other diseases with cutaneous involvement: leucocytoclastic vasculitis, Wegener’s granulomatosis [5]and systemic PAN [2, 3, 4]. In the 3 cases of patients with cutaneous PAN successfully treated with a low-dose MTX therapy [1], the maximum dose of MTX required was 10, 15, and 25 mg/week.…”

Section: Discussionmentioning

confidence: 99%

“…There are arguments for tumour necrosis factor (TNF)-α and interleukin (IL)-1 to be involved in systemic PAN [4, 6]: (1) transcription of the TNF-α gene in peripheral blood monocyte cells from patients with systemic vasculitis is enhanced; (2) IL-1 activates endothelial cells to express adhesion molecules and to produce IL-6 and IL-8 that stimulate the chemotactic activity of neutrophils. Coll-Vinent et al [7]showed that constitutive and inducible adhesion molecules, e.g.…”

Section: Discussionmentioning

confidence: 99%

“…MTX was shown in vitro to suppress the activity of TNF. It inhibits IL-1β binding to its cell surface receptors [4], probably in a competitive fashion with a secreted IL-1 receptor antagonist, as neither IL-1 synthesis nor secretion is altered [9]. Moreover, a reduced ICAM-1 and VCAM-1 expression in synovial tissue in patients with active rheumatoid arthritis was noted after 4 months of treatment [10].…”

Section: Discussionmentioning

confidence: 99%

“…As far as we know, only 3 patients with cutaneous PAN [1]and about 3 cases of systemic PAN [2, 3, 4]treated successfully with MTX have been reported in the medical literature until now. We report 2 further cases of patients affected by a steroid-dependent cutaneous PAN that regressed with weekly low-dose MTX therapy.…”

Section: Introductionmentioning

confidence: 99%

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Successful Treatment in Two Cases of Steroid-Dependent Cutaneous Polyarteritis nodosa with Low-Dose Methotrexate

et al. 2001

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To the best of our knowledge, only 3 cases of cutaneous polyarteritis nodosa (PAN) treated successfully with methotrexate (MTX) have been reported in the medical literature. We report 2 further cases of steroid-dependent cutaneous PAN treated successfully with low-dose weekly MTX therapy. The clinical and biological tolerance of MTX was excellent. The cutaneous lesions started to regress within 3 weeks. One of the patients reported full recovery which lasted 2 years after stopping the therapy. So, MTX seems to be an interesting therapy in the treatment of PAN because of its relatively low toxicity, its simple use, its quick action and prolonged results after MTX has been stopped.

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