shrinking lung syndrome

[hihannula]

Has anyone else been diagnosed with a rare condition called shrinking lung syndrome. It’s so rare, approximately only 0.5-1.1 % people with Lupus get it. I’m real scared as at time I feel like I’m slowly suffocating! Still getting more tests done to see if diaphragm isn’t partially paralyzed. Did you know, you could be in full lupus remission and the Shrinking Lung syndrome can stay activated! So frightening!😰😥

[lupus-support1 Administrator]

Yes. In fact, I was diagnosed with “Lupus Lung” before Dr Graham Hughes diagnosed me with lupus.

First & foremost: don’t panic. It’s not a terminal condition. It’s benign. It sometimes accompanies pain on breathing. It’s also called “shrinking lung” syndrome, which causes a sense of breathlessness and the feeling that the lungs are not able to expand.

Note: it’s a feeling and sometimes we can panic, which worsens the situation. Sometimes an asthma inhaler can help because it expands the lungs. Asthma inhalers can be steroids or non-steroids. But, it’s important to see an expert who can help you learn how to “breathe” when you are symptomatic. For example, slow, deep breaths. Sometimes the intercostals muscle will be painful ( what isn’t painful in lupus?), and an X-ray is important.

Shrinking lung syndrome is a rare complication of systemic autoimmune disease, most commonly SLE. This condition presents with exertional dyspnoea with or without pleuritic chest pain. It is rare, affecting approximately 1% of patients with SLE, and can occur at any point in the disease. In those patients with radiographic evidence of an elevated hemidiaphragm and restrictive physiology on lung function testing in the absence of another cause, it should be suspected. Initial treatment is usually with corticosteroids. More intense immunosuppression with disease-modifying and biologic drugs is sought in refractory cases. Most patients improve with treatment.

Prognosis

Shrinking lung syndrome has a good prognosis. Most patients achieve some improvement with appropriate immunosuppressive therapy. Clinical improvement is common, with most patients reporting symptomatic relief. There is stabilization or improvement in lung function testing in most cases.

My advice: go and see an expert in lung diseases. I was lucky in seeing one who also knew about SLE, because he had worked with Dr Graham Hughes!

I hope this is helpful.

Will good wishes,

Ros