Hypocomplementemic Urticarial Vasculitis (HUVS) with Precocious Emphysema Responsive to Azathioprine

Breda, Luciana; Nozzi, Manuela; Harari, Sergio; Torto, Marianna Del; Laganà, Marta; Sereno, Alessandra; Chiarelli, Francesco

doi:10.1007/s10875-013-9886-1

Cited by 8 publications

(7 citation statements)

References 17 publications

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“…Features include low serum complement and urticarial symptoms lasting longer than 6 months, with vasculitic findings on skin biopsy. Spirometry findings consistent with obstructive lung disease have been reported in the case of a teenager who later developed exertional dyspnoea and emphysematous changes on CT of the thorax [31].…”

Section: Hypocomplementemic Urticarial Vasculitissupporting

confidence: 59%

Pulmonary manifestations of systemic vasculitis in childhood

Lawton

Machta

Semple

et al. 2020

Breathe

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The systemic vasculitides are a heterogenous group of rare conditions with an incompletely understood aetiology. Any of the systemic vasculitides may cause respiratory disease, but some conditions are more likely to affect the pulmonary system, often through pulmonary infarction and diffuse alveolar haemorrhage. These conditions are often difficult to diagnose due to their rarity and significant clinical overlap with common respiratory conditions. Prompt diagnosis and management can significantly reduce morbidity and mortality.

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Section: Hypocomplementemic Urticarial Vasculitissupporting

confidence: 59%

Pulmonary manifestations of systemic vasculitis in childhood

Lawton

Machta

Semple

et al. 2020

Breathe

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“…Time to reach partial/total remission in UV differs among various treatment modalities. Azathioprine achieved a dramatic resolution in symptoms within 18 months in a patient with HUV 3 . Worm et al reported a case of HUV successfully treated with cyclophosphamide‐dexamethasone pulse therapy given every 2 weeks, clinical remission was established after the 10th pulse 4 .…”

Section: Figurementioning

confidence: 99%

Rapid response to combination of hydroxychloroquine and prednisolone in a patient with refractory hypocomplementemic urticarial vasculitis

Bostan

Akdoğan

Gököz

et al. 2020

Dermatologic Therapy

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“…In the chronic and resistant subset of patients, corticosteroid-sparing immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine A, or mycophenolate mofetil have been shown to be effective [4]. Azathioprine has been shown to be a useful adjunct to corticosteroids for stabilization of renal and pulmonary function [4,68]. Methotrexate has usually inconsistent and disappointing results in the treatment of UV [4,24,33,69].…”

Section: Treatmentmentioning

confidence: 99%

Urticarial Vasculitis

Koç¹,

Aksoy²,

Tatlıparmak³

2017

A Comprehensive Review of Urticaria and Angioedema

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Urticarial vasculitis (UV) is a small vessel vasculitis and an immune-complex mediated disease like other leukocytoclastic vasculitis. UV seems similar to common urticaria clinically. Major difference between urticarial vasculitis and urticaria is the duration of lesions. Urticarial lesions regress in 24 hours, but UV lesions persist longer than 24 hours. Residual hyperpigmentation, constitutional symptoms like fever, arthralgia, and abdominal pain are other main clinical differences between these disorders. Upon confirmation of diagnosis, patients are divided into two major categories on the basis of serum complement levels: normocomplementemic UV (NUV) and hypocomplementemic UV (HUV). Consensus meeting in 1996 stated that long lasting (at least 24 hour-5 days) indurated wheals, which may be itchy, painful or tender, be associated with purpura and presence of associated extracutaneous findings, and cutaneous vasculitis confirmed by histopathological examination are defined as UV.

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Hypocomplementemic Urticarial Vasculitis (HUVS) with Precocious Emphysema Responsive to Azathioprine

Cited by 8 publications

References 17 publications

Pulmonary manifestations of systemic vasculitis in childhood

Pulmonary manifestations of systemic vasculitis in childhood

Rapid response to combination of hydroxychloroquine and prednisolone in a patient with refractory hypocomplementemic urticarial vasculitis

Urticarial Vasculitis

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