Hamman-Rich syndrome

Hamman-Rich syndrome is a rare and severe form of interstitial lung disease (ILD) that progresses rapidly, often leading to respiratory failure and death. It is also known as acute interstitial pneumonia (AIP).

Etiology

The exact cause of Hamman-Rich syndrome is unknown. However, it is believed to be an inflammatory response to an unknown trigger, possibly a viral infection or an environmental exposure.

Symptoms

The symptoms of Hamman-Rich syndrome typically develop rapidly, over a period of days to weeks. They may include shortness of breath, cough, fatigue, weight loss, and fever. As the disease progresses, patients may develop respiratory failure and require mechanical ventilation.

Diagnosis

Diagnosis of Hamman-Rich syndrome is challenging due to its rarity and the lack of specific diagnostic criteria. It is often diagnosed based on the clinical presentation and the exclusion of other causes of ILD. Imaging studies, such as a CT scan, may show diffuse ground-glass opacities in the lungs. A lung biopsy may be necessary to confirm the diagnosis.

Treatment

There is no specific treatment for Hamman-Rich syndrome. Management is primarily supportive, including oxygen therapy and mechanical ventilation if necessary. Some patients may benefit from corticosteroids or other immunosuppressive drugs, although the evidence for their effectiveness is limited.

Prognosis

The prognosis for Hamman-Rich syndrome is generally poor, with a high mortality rate. Many patients die within months of diagnosis due to respiratory failure. However, some patients may have a more indolent course and survive for several years.

See also

• Idiopathic pulmonary fibrosis
• Acute respiratory distress syndrome

NIH genetic and rare disease info

• NIH info on Hamman-Rich syndrome

Hamman-Rich syndrome is a rare disease.

Hamman-Rich syndrome Resources
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