A 61-year-old woman came to our practice for a second opinion after her nearly constant cough prompted a CAT scan that revealed nodules all over her lungs.
She never smoked, and couldn’t identify any allergens or toxic exposures that could be causing her terrible cough.
The problem had been developing for a long time. For more than 20 years she complained of a dry cough and shortness of breath. By now, she could not take a deep breath, raise her voice, or sing without triggering an unbearable coughing fit.
For years, she had been treated for allergic rhinitis, or hay fever, and cough-variant asthma, which is a type of asthma characterized by a dry, non-productive cough. But none of the treatments seemed to help.
Finally, she had a CAT scan to screen for coronary artery disease. What it revealed however, were so many nodules that it was feared she might have metastatic lung cancer.
Pulmonary function testing suggested she had obstructive lung disease. Another CAT scan of the chest confirmed her many pulmonary nodules. Lung biopsies revealed tiny clusters of cells that release hormones into the blood.
What was causing her constellation of symptoms?
To her great relief, our patient did not have cancer.
Instead, her years of respiratory symptoms, chronic cough, and test results led us to diagnose a rare lung disease known as diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. DIPNECH is caused by hormone-secreting cells in the lungs that lead to pulmonary nodules and chronic respiratory symptoms that are notoriously tough to explain.
While there is no standardized treatment for this condition, some centers have had success with therapies that counter the hormones produced by the cells, slowing progression of the disease. In most cases, the disease does not get worse, and many patients require no therapy at all.
Gender bias in medicine, however unintended, has a hand in explaining why our patient had such a hard time finding out what was wrong. For instance, while more women die with COPD than men, physicians are still more likely to correctly diagnose men with this life-threatening condition. Similarly, more men are diagnosed with lung cancer each year even though the risk that a lung nodule is malignant is greater in women than in men.
DIPNECH, which is found almost exclusively in middle-aged women who are nonsmokers, is another example. It took a multidisciplinary approach and careful consideration of her long-standing symptoms to correctly diagnose our patient, whose prognosis now is good.
She is thrilled that she no longer needs to take steroids or inhalers prescribed for conditions she didn’t even have and that didn’t help her. Without the steroids, she finds it easier to keep her weight down and she is able to exercise more regularly. Her shortness of breath and cough are greatly improved.
When we first met her, she was afraid she wouldn’t live to see her daughter’s wedding. Now she is waiting for the birth of her first grandchild in June.
Jamie Garfield, MD, is an associate professor of thoracic medicine and surgery at Lewis Katz School of Medicine at Temple University, and assistant director of quality improvement and core faculty for the Internal Medicine Residency Program at Temple University Hospital.