Severe panniculitis caused by homozygous ZZ alpha1-antitrypsin deficiency treated successfully with human purified enzyme (ProlastinR)

Chowdhury, M. M. U.; Williams, Evan J.; Morris, John; Ferguson, Berrylin J.; McGregor, A.D.; Hedges, Allan R.; Stamatakis, J. D.; Pope, F M

doi:10.1046/j.1365-2133.2002.05095.x

Cited by 46 publications

(21 citation statements)

References 5 publications

(6 reference statements)

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“…Several case studies have revealed that infusion of a higher dose of AAT augmentation therapy compared to that required for the treatment of lung disease, successfully managed and reduced inflammation associated with severe panniculitis in AATD individuals with the ZZ phenotype (Al-Niaimi and Lyon 2011; Blanco et al 2011;Chowdhury et al 2002;Dowd et al 1995;Gross et al 2009). The prevalence of panniculitis as a complication of AATD is infrequent, with an occurrence of approximately 0.1%.…”

Section: The Use Of Aat Augmentation Therapy In Treatment Of Panniculmentioning

confidence: 99%

Alpha-1 Antitrypsin: A Potent Anti-Inflammatory and Potential Novel Therapeutic Agent

Bergin

Hurley

McElvaney

et al. 2012

Arch. Immunol. Ther. Exp.

119

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Alpha-1 antitrypsin (AAT) has long been thought of as an important anti-protease in the lung where it is known to decrease the destructive effects of major proteases such as neutrophil elastase. In recent years, the perception of this protein in this simple one dimensional capacity as an anti-protease has evolved and it is now recognised that AAT has significant anti-inflammatory properties affecting a wide range of inflammatory cells, leading to its potential therapeutic use in a number of important diseases. This present review aims to discuss the described anti-inflammatory actions of AAT in modulating key immune cell functions, delineate known signalling pathways and specifically to identify the models of disease in which AAT has been shown to be effective as a therapy.

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Section: The Use Of Aat Augmentation Therapy In Treatment Of Panniculmentioning

confidence: 99%

Alpha-1 Antitrypsin: A Potent Anti-Inflammatory and Potential Novel Therapeutic Agent

Bergin

Hurley

McElvaney

et al. 2012

Arch. Immunol. Ther. Exp.

119

View full text Add to dashboard Cite

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“…Appropriate histology and serum concentration of AAT below 30% of the lower reference value prior to the treatment further support the diagnosis of AAT deficiency-related wound healing impairment. Successful augmentation therapy of AAT deficiency-associated panniculits has been described earlier (21). While other cases reported in the literature were treated with either tetracycline, steroids, non-steroidal anti-inflammatory drugs or hydroxychloroquine, individualised Prolastin ® therapy in terms of starting time of application and dosage showed to be the most effective treatment strategy (22).…”

Section: Resultsmentioning

confidence: 97%

Severe postoperative wound healing disturbance in a patient with alpha‐1‐antitrypsin deficiency: the impact of augmentation therapy

Cathomas

Schüller

Candinas

et al. 2015

International Wound Journal

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Wound healing disturbance is a common complication following surgery, but the underlying cause sometimes remains elusive. A 50-year-old Caucasian male developed an initially misunderstood severe wound healing disturbance following colon and abdominal wall surgery. An untreated alpha-1-antitrypsin (AAT) deficiency in the patient's medical history, known since 20 years and clinically apparent as a mild to moderate chronic obstructive pulmonary disease, was eventually found to be at its origin. Further clinical work-up showed AAT serum levels below 30% of the lower reference value; phenotype testing showed a ZZ phenotype and a biopsy taken from the wound area showed the characteristic, disease-related histological pattern of necrotising panniculitits. Augmentation therapy with plasma AAT was initiated and within a few weeks, rapid and adequate would healing was observed. AAT deficiency is an uncommon but clinically significant, possible cause of wound healing disturbances. An augmentation therapy ought to be considered in affected patients during the perioperative period.

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“…Many clinicians regard dapsone as the initial treatment of choice, based on modest efficacy and reasonable expense. At the same time, though described in only 2 reports to date [19, 33], intravenous augmentation therapy has consistently been associated with prompt resolution of the signs and symptoms of AAT deficiency-associated panniculitis. Reports to date have described conventional doses of 60 mg/kg body weight once weekly, which conferred only incomplete benefit in our patient.…”

Section: Discussionmentioning

confidence: 99%

New Findings in PiZZ α<sub>1</sub>-Antitrypsin Deficiency-Related Panniculitis

et al. 2009

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Panniculitis is a recognized but unusual complication of a severe deficiency of α₁-antitrypsin (AAT), with fewer than 100 cases described to date. Like the pathogenesis of emphysema in severe PiZZ deficiency of AAT, panniculitis has been hypothesized to be an inflammatory process, possibly related to Z AAT polymer formation and to an unopposed anti-inflammatory screen in the context of deficient serum levels of AAT. The current report presents a 31-year-old woman with PiZZ AAT deficiency-associated panniculitis. Our case extends current knowledge of AAT-associated panniculitis in 2 ways: (1) we demonstrate Z-type AAT polymers in the skin, which supports the inflammatory pathogenesis of panniculitis and the potential pro-inflammatory role of polymers; (2) we show that a high dose and long-term use of intravenous augmentation therapy (90 mg/kg body weight once weekly during 3 years) can ameliorate the frequency and severity of panniculitis associated with AAT deficiency.

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Severe panniculitis caused by homozygous ZZ alpha1-antitrypsin deficiency treated successfully with human purified enzyme (ProlastinR)

Cited by 46 publications

References 5 publications

Alpha-1 Antitrypsin: A Potent Anti-Inflammatory and Potential Novel Therapeutic Agent

Alpha-1 Antitrypsin: A Potent Anti-Inflammatory and Potential Novel Therapeutic Agent

Severe postoperative wound healing disturbance in a patient with alpha‐1‐antitrypsin deficiency: the impact of augmentation therapy

New Findings in PiZZ α<sub>1</sub>-Antitrypsin Deficiency-Related Panniculitis

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