Hypocomplementaemic urticarial vasculitis: successful treatment with cyclophosphamide-dexamethasone pulse therapy

Worm, Margitta; Muche, Marion; Schulze, P; Sterry, Wolfram; Kolde, G.

doi:10.1046/j.1365-2133.1998.02473.x

Cited by 40 publications

(14 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Cyclophosphamide with pulse dexamethasone has been used successfully in unresponsive HUV [33], and mycophenolate mofetil has been a useful maintenance therapy [33,34]. Weekly rituximab therapy was successful in a patient with HUVS who had a temporary response to intravenous immunoglobulin (IVIG) [35].…”

Section: (Continued)mentioning

confidence: 99%

Urticarial vasculitis

Brown

Carter²

2007

Curr Rheumatol Rep

View full text Add to dashboard Cite

Urticarial vasculitis can present in a variety of ways, ranging from a primarily cutaneous disease consisting of chronic urticaria to a lupus-like disease with severe cardiopulmonary disease. Low complement levels and positive anti-C1q antibodies are markers of more severe disease. Care must be taken to look for an underlying condition. The mainstay of therapy is treatment of any underlying condition. Therapies most often employed include corticosteroids, antihistamine, and dapsone, but many others have been utilized.

show abstract

Section: (Continued)mentioning

confidence: 99%

Urticarial vasculitis

Brown

Carter²

2007

Curr Rheumatol Rep

View full text Add to dashboard Cite

show abstract

“…In regard to CSVV, Worm et al [34] described two patients with UV with limited systemic involvement (ie, arthritis and episcleritis or conjunctivitis), which was refractory to traditional therapies (eg, prednisone, dapsone and pentoxifylline, IVIG, azathioprine, and cyclosporine), who responded to intravenous dexamethasone-cyclophosphamide (100 mg dexamethasone, 500 mg cyclophosphamide) for 3 days every 2 weeks. Patients received 50 mg of oral cyclophosphamide between pulses.…”

Section: Cyclophosphamidementioning

confidence: 98%

Primary cutaneous small vessel vasculitis

Russell

Weenig

2004

Curr Treat Options Cardio Med

View full text Add to dashboard Cite

Disorders associated with cutaneous vasculitis include numerous well-described etiologies. Primary cutaneous vasculitis limits discussion to primary leukocytoclastic vasculitis, essential mixed cryoglobulinemia, urticarial vasculitis, Henoch-Schönlein purpura, and erythema elevatum diutinum. Although the therapeutics for these disorders are based on limited data, we attempt to construct a consensus opinion on the management of primary cutaneous vasculitis. Therapy of primary cutaneous vasculitis is indicated for symptomatic or systemic involvement, because cutaneous small vessel vasculitis is frequently a self-limited, single episodic disease. Conservative, symptomatic treatment includes leg elevation, warming, antihistamines, and nonsteroidal anti-inflammatory drugs. For mild recurrent disease, colchicine, dapsone, and prednisone are first-choice agents. Systemic or severe cutaneous disease requires more potent immunosuppression (eg, prednisone, azathioprine, or mycophenolate mofetil). Plasmapheresis/plasma exchange and intravenous immunoglobulin are viable considerations for refractory disease, but are cumbersome and expensive modalities. There is insufficient evidence to advocate the use of new biological or monoclonal antibody therapies in primary cutaneous vasculitis.

show abstract

“…Cyclophosphamide and dexamethasone pulse therapy have been used in cases of refractory disease [38]. Triple therapy with glucocorticoids, dapsone, and cyclophosphamide in resistant cases of HUVS may be necessary.…”

Section: Severe or Refractory Systemic Diseasementioning

confidence: 99%

Hypocomplementemic urticarial vasculitis syndrome

Jara

Navarro²,

Medina³

et al. 2009

Curr Rheumatol Rep

View full text Add to dashboard Cite

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an uncommon immune complex-mediated entity characterized by urticaria with persistent acquired hypocomplementemia. First described in 1973, HUVS is associated with several systemic findings including leukocytoclastic vasculitis, severe angioedema, laryngeal edema, pulmonary involvement, arthritis, arthralgia, glomerulonephritis, and uveitis. These manifestations should be present for at least 6 months. Laboratory findings include low complement levels of classical pathway, namely C1q, C2, C3, and C4. The disease marker is the serum presence of anti-C1q antibodies. Treatment, based on disease severity, involves corticosteroids and other immunosuppressive agents that have demonstrated some success. Patients may have significant morbidity and mortality, most commonly caused by chronic obstructive pulmonary disease and acute laryngeal edema.

show abstract

Hypocomplementaemic urticarial vasculitis: successful treatment with cyclophosphamide-dexamethasone pulse therapy

Cited by 40 publications

References 9 publications

Urticarial vasculitis

Urticarial vasculitis

Primary cutaneous small vessel vasculitis

Hypocomplementemic urticarial vasculitis syndrome

Contact Info

Product

Resources

About