shop helvetica, drugs sans-serif; font-size: 1em; background-color: #92d2dd; border: medium solid #333333; text-align: center;">Pulmonary complications are the leading cause of danger to patients with ataxia telangiectasia, pills ahead of cancer.

The immune problems are largely the cause.

Research is progressing rapidly to treatment.

You, parents, can make prevention.



The term "pneumonia" refers to lung diseases affecting the deep lung.
The bronchi may also be sick from immune deficiency and recurrent bronchitis, with the development of bronchiectasis (permanent bronchi expansion).

We are talking about:

Lungs and immunity

We saw in chapter "Immunity and AT" that people with AT have a combined immunodeficiency, variable according to individuals, which affects both:

Even the mucous membranes (the first barriers) are affected in ataxia telangiectasia (accelerated aging, IgA deficiency). Those of the respiratory system are therefore entry points for frequent and dangerous infections. They are favored by:

The deficit in T cells (the main actors of the specific -with memory- immune response ), which promotes:

The deficit in B cells and immunoglobulins, which promotes:

These deficits may partly be made up by IV of immunoglobulin, but they do not cover the full spectrum of viruses and bacteria. To do this, and according to the deficit, the doctor may prescribe regular and permament medication to a particular antibiotic.


These infections pose a risk of lightning extension and respiratory distress syndrome, irreversible if not caught in time. Even if remission, sequelae may remain with the development of bronchiectasis.

Thumbnail imageBronchiectasis
It is the result of repeated lung infections. It is the chronic inflammation and airway obstruction by secretions that damage the bronchial wall.

Preventive treatment is based on immunoglobulin and antibiotics for infections. If bronchiectasis is established, treatment is based on chest physiotherapy (to help drainage of congested airways), in addition to preventive treatment that must be pursued.

This treatment is essential to slow the worsening of the lesions. Indeed, bronchiectasis, once installed, creates a vicious circle:

The clinical implications are:

Thumbnail imageInterstitial pneumonia
Patients with AT may also develop an interstitial pneumonia (see definition at the beginning). It would be the consequence of abnormal lung repair facing the various assaults by deregulation of the immune system or lack of healing after lung aggression (infections, chemical oxidants ...).

The alveoli and the supporting tissue of these cells that are damaged. The final stage is fibrosis with consequent decrease in breathing capacity and a failure to transfer oxygen from the lungs into the blood. This complication is relatively rare (about 5% of patients) but serious, therefore it requires regular pneumologic monitoring to detect and treat it early.


In the bleak picture of ataxia telangiectasia, pulmonary infections are the leading cause of mortality.

But paradoxically, they are also one of the few points on which the family circle can be influential(cf "pneumologist"). In two words: vigilance and prevention.

Moreover, if there are still many unknowns about how these infections destroy the lungs, investigation techniques are in constant progress and treatment are increasingly powerful.