From the Respiratory Research Division, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin, Ireland.
From the Respiratory Research Division, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin, Ireland..
Chest. 2015 Nov;148(5):e136-e138. doi: 10.1378/chest.15-0699.
Panniculitis associated with α1-antitrypsin deficiency (AATD) is well documented but rare. We report the first case, to our knowledge, of successful induction of clinical remission of AATD-related panniculitis following a single 120-mg/kg dose administration of plasma-purified α1-antitrypsin (AAT). A 23-year-old man with known PiZZ AATD presented to the hospital with a diffusely swollen and tender right upper limb. This was associated with subcutaneous induration, and a discrepancy of 5 cm in upper limb circumference at the mid arm was noted. There was no convincing precipitant for cellulitis or an infectious cause, and inflammatory markers were raised, with a C-reactive protein (CRP) level of 93.9 mg/L and erythrocyte sedimentation rate (ESR) of 71 mm/h. Doppler ultrasonography ruled out DVT. No antimicrobials or antiinflammatory medications were administered during or prior to admission. Biopsy specimens of the right upper limb revealed extensive panniculitis with neutrophils, foamy macrophages, and fat necrosis. A diagnosis of AATD-associated panniculitis was made. Following this, a single IV dose of 120 mg/kg of plasma-purified AAT was administered. By day 7 post AAT infusion, CRP level had normalized to 4.6 mg/L and ESR had dropped to 22 mm/h. Limb circumference discrepancy on day 7 was 1 cm. There was no tenderness to palpation or induration, and a clinical remission of panniculitis was observed. We report the first case, to our knowledge, of clinical remission following a single treatment with IV AAT at a dose of 120 mg/kg. This opens avenues to more timely and effective treatment of the more severe presentations of AAT-associated panniculitis.
与α1-抗胰蛋白酶缺乏症（AATD）相关的脂膜炎已有大量文献记载，但较为罕见。我们报告首例已知的 PiZZ 型 AATD 患者，在接受单次 120mg/kg 剂量的血浆纯化α1-抗胰蛋白酶（AAT）治疗后，成功诱导 AATD 相关脂膜炎的临床缓解。一位 23 岁男性，已知患有 PiZZ 型 AATD，因右上肢弥漫性肿胀和触痛就诊。该患者还伴有皮下硬结，上臂中部的上肢周径差异为 5cm。没有明确的蜂窝织炎或感染性病因，炎症标志物升高，C 反应蛋白（CRP）水平为 93.9mg/L，红细胞沉降率（ESR）为 71mm/h。多普勒超声检查排除了深静脉血栓形成。在入院期间或之前，未使用任何抗生素或抗炎药物。右上肢活检标本显示广泛的伴有中性粒细胞、泡沫状巨噬细胞和脂肪坏死的脂膜炎。诊断为 AATD 相关脂膜炎。此后，给予患者单次 120mg/kg 的静脉注射 AAT。在接受 AAT 输注后第 7 天，CRP 水平正常化至 4.6mg/L，ESR 降至 22mm/h。第 7 天的肢体周长差异为 1cm。触诊时无压痛或硬结，脂膜炎的临床缓解得到观察。我们报告首例已知的 PiZZ 型 AATD 患者，在接受单次 120mg/kg 剂量的 IV AAT 治疗后，临床缓解。这为更及时、更有效的治疗 AAT 相关脂膜炎更严重的表现开辟了道路。
