Clinical features of patients with diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
| Clinical features | Group 1 (n = 9) (Symptomatic) | Group 2 (n = 10) (Asymptomatic) | Overall (n = 19) |
|---|---|---|---|
| DIPNECH, diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. | |||
| n = number of patients for whom data were available. | |||
| *Symptoms felt due to other disease rather than DIPNECH. | |||
| †Does not include immunosuppression after transplantation (n = 1, group1), chemotherapy for carcinoma (n = 2, group 2) and chemotherapy for metastatic atypical carcinoid (n = 1, group 2). | |||
| ‡One patient died of chronic rejection after transplantation. | |||
| §One patient died of carcinoma of the large bowel at 5 years. | |||
| Male : female (n = 19) | 2 : 7 | 2 : 8 | 4 : 15 |
| Smoking history (n = 17) (never: ex-smoker : current) | 5 : 2 : 1 | 7 : 2 : 0 | 12 : 4 : 1 |
| Mean age at onset (years) | 49.1 (31–67) | NA | |
| Presenting complaints: | |||
| Cough | 4/9 | 0/10 | 4/19 |
| Increasing dyspnoea | 6/9 | 0/10 | 6/19 |
| Pleuritic chest pain | 2/9 | 0/10 | 2/19 |
| Haemoptysis | 1/9 | 1/10* | 2/19 |
| Asymptomatic | 0/9 | 9/10* | 9/19* |
| Previous malignancy | 0/9 | 8/10 | 8/19 |
| History of asthma | 3/9 | 2/10 | 5/19 |
| Lung function (n = 16; obstructive : mixed : normal) | 5 : 3: 0 | 3: 0: 5 | 8 : 3: 5 |
| Mean duration of illness before diagnosis (years) | 8.6 | NA | 8.6 |
| Bronchoalveolar lavage | Lymphocytosis 2/2 | – | Lymphocytosis 2/2 |
| Treatment† | |||
| Steroids | 2/6 | 0/8 | 2/14 |
| Watch and wait | 3/6 | 4/8 | 7/14 |
| Follow-up | |||
| Clinically stable (alive with disease) | 5/7 (range 1–12 years, mean 5.8 years) | 6/7 (range 0.2–2.2 years, mean 1.0 year) | 11/14 |
| Clinical deterioration | 1/7 | 0/7 | 1/14 |
| Died of DIPNECH | 1/7‡ | 0/7 | 0/14 |
| Died of other disease | 0/7 | 1/7§ | 1/14 |