A 21-year-old woman with cystic fibrosis (CF) was seen in the pulmonary clinic complaining of abdominal pain. Her past medical history included bilateral lung transplantation for CF pulmonary disease 26 months previously, as well as gastroesophageal reflux disease and pancreatic insufficiency. Her baseline weight was 49.1 kg (BMI, 19.4 kg/m2).

A 23-year-old white man was admitted to the hospital for evaluation of recurrent hemoptysis. He denied any other associated symptoms, including dyspnea, chest pain, productive cough, wheezing, fever, or weight loss. He had no significant past medical history and was not taking any medication. He had no significant family history for cardiopulmonary diseases.

A 34-year-old woman presented with her third episode of acute-onset right-sided chest pain and dyspnea. She had two prior similar occurrences of right-sided sharp, pleuritic chest pain with radiation to the back and dyspnea. Chest radiographs during these presentations revealed a small apical right-sided pneumothorax that was managed conservatively with high-flow oxygen. All three presentations were associated with vigorous exercise and the first day of her menses. She denied cough, hemoptysis, fever, smoking history, airplane travel, scuba diving, or trauma during these presentations. The patient has been trying to conceive for the past year but has been unsuccessful because of uterine fibroids but no history of endometriosis.

A 66-year-old male nonsmoker from Arizona was referred to our practice for evaluation of chronic cough. He had a history of biopsy-proven relapsing polychondritis manifesting as right auricular and nasal pain and swelling 9 months prior to presentation. The onset of his cough coincided with the diagnosis of relapsing polychondritis, and he was prescribed prednisone 90 mg/d, which promptly relieved his rheumatologic and respiratory symptoms. A chest radiograph, obtained prior to the initiation of therapy, was normal. Any attempts at decreasing the dose of the glucocorticoid to < 30 mg/d resulted in recurrence of the cough but not of the auricular or nasal symptoms. A second chest radiograph done 6 months before presentation, while the patient was receiving prednisone 20 mg/d, was normal as well. In anticipation of our evaluation, he stopped all glucocorticoids for 7 days. He was not receiving any other medications, and he had no history of an atopic diathesis.

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.