A 39-year-old woman presented with a 6-month history of breathlessness, productive cough and painful lumps on her lower legs. She had suffered with recurrent rhinosinusitis and frequent infections of the ears, throat and chest since childhood, as well as cutaneous herpes zoster in her 20s. There was no documented history of pneumonia, invasive or deep-seated infections. She had smoked lightly, was allergic to penicillin and worked as a commercial tea buyer. Examination showed a slim female with a normal breathing pattern. Chest expansion was reduced and fine inspiratory crackles were detected over the lower lung zones. Pulmonary function tests showed ventilatory restriction with decreased gas transfer. Forced expiratory volume in one second (FEV1) 2.10 L, 71% predicted; forced vital capacity (FVC) 2.22 L, 65% predicted; spirometric ratio 0.93; total lung capacity 3.04 L, 62% predicted; haemoglobin (Hb)-adjusted carbon monoxide transfer factor/TLco 45% predicted and its coefficient/Kco 81% predicted. Indices related to small airway function were normal, maximal expiratory flow (MEF)75/50/25 all above 80% predicted and air-trapping was absent. Bloods tests showed normal full blood count, C-reactive protein as well as kidney, bone and liver biochemistry. Serum ACE was elevated at 93 ACEU (ref: 12–68). Elispot assay and HIV serology were negative. A prereferral chest radiograph revealed widening of the superior mediastinum and ill-defined nodular opacities in the middle and lower zones. Despite the history of possible erythema nodosum and raised serum ACE, the account of recurrent infections suggested a differential diagnosis broader than typical sarcoidosis. Demonstration of ventilatory restriction and impaired gas transfer factor hinted towards an interstitial or diffuse lung pathology. The Kco, at nearly twice the TLco in %-predicted terms, indicated a low likelihood of major pulmonary vasculopathy and the absence of airflow limitation or small airway dysfunction steered the diagnosis away from primary bronchiolar disorders. CT with contiguous …

中文翻译：

---

弥漫性肉芽肿病：肺内外看

一名 39 岁女性因呼吸困难、咳嗽咳痰和小腿疼痛肿块 6 个月就诊。她从小就患有反复鼻窦炎，耳朵、喉咙和胸部经常感染，20多岁时还患了皮肤带状疱疹。没有记录在案的肺炎、侵袭性或深部感染病史。她抽过一点烟，对青霉素过敏，并且是一名商业茶叶采购员。检查显示一名身材苗条的女性，呼吸模式正常。胸部扩张减少，在下肺区检测到细微的吸气爆裂音。肺功能测试显示通气受限，气体转移减少。一秒用力呼气量 (FEV1) 2.10 L，预测为 71%；用力肺活量 (FVC) 2.22 L，65% 预测值；肺活量比 0.93；总肺活量 3.04 L，预计为 62%；血红蛋白 (Hb) 调整的一氧化碳转移因子/TLco 预测值 45% 及其系数/Kco 预测值 81%。与小气道功能相关的指数正常，最大呼气流量 (MEF)75/50/25 均高于预测值的 80%，并且不存在空气滞留。血液检查显示全血细胞计数、C 反应蛋白以及肾脏、骨骼和肝脏生物化学均正常。血清 ACE 升高到 93 ACEU（参考：12-68）。Elispot 检测和 HIV 血清学检测均为阴性。转诊前胸片显示上纵隔增宽和中下部区域不明确的结节影。尽管可能有结节性红斑和血清 ACE 升高的病史，但反复感染的原因表明鉴别诊断比典型的结节病更广泛。通气受限和气体转移因子受损的证明暗示存在间质性或弥漫性肺病变。Kco 几乎是 TLco 的 % 预测值的两倍，表明主要肺血管病变的可能性很低，并且没有气流受限或小气道功能障碍使诊断远离原发性细支气管疾病。CT与连续…