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Case based discussions
Diffuse granulomatous disease: looking inside and outside the lungs
  1. Alan Williams1,
  2. W Peter Kelleher2,
  3. Andrew G Nicholson3,
  4. Anand Devaraj4,
  5. Carlos Pavesio5,
  6. Felix Chua1
  1. 1 Interstitial Lung Disease, Royal Brompton and Harefield NHS Foundation Trust, London, UK
  2. 2 Immunology, Imperial College NHS Trust, London, UK
  3. 3 Department of Histopathology, Royal Brompton and Harefield NHS Foundation Trust, London, UK
  4. 4 Department of Radiology, Royal Brompton and Harefield NHS Foundation Trust, London, UK
  5. 5 Moorfields Eye Hospital NHS Foundation Trust, London, UK
  1. Correspondence to Dr Alan Williams, Interstitial Lung Disease, Royal Brompton Hospital, London SW3 6NP, UK; alanw{at}doctors.org.uk

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Dr Alan Williams

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.

Dr Felix Chua

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 …

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