HR syndrome
Classification & external resources

ICD-10	J84.1
ICD-9	516.3
OMIM	178500
DiseasesDB	4815

Hamman-Rich syndrome (also known as acute interstitial pneumonia) is a rare, severe lung disease which usually affects otherwise healthy individuals.

As of 2005, there is no known cause or treatment.

HR syndrome is often categorized as both an interstitial lung disease and a form of acute respiratory distress syndrome (ARDS).

Symptoms

The most common symptoms of Hamman-Rich syndrome are cough, fever, and difficulties breathing. These often occur over a period of one to two weeks before medical attention is sought. Difficulties breathing can quickly progress to an inability to breathe without support (respiratory failure).

Hamman-Rich syndrome typically progresses rapidly, with hospitalization and mechanical ventilation often required only days to weeks after initial symptoms of cough, fever, and difficulties breathing develop.

Diagnosis

Rapid progression from initial symptoms to respiratory failure is a key feature of Hamman-Rich syndrome. An x-ray which shows ARDS is necessary for diagnosis (fluid in the small air sacs (alveoli) in both lungs). In addition, a biopsy of the lung which shows organizing diffuse alveolar damage is required for diagnosis. Other diagnostic tests are useful excluding other similar condition, but history, x-ray, and biopsy are necessary. These other tests may include basic blood work, blood cultures, and bronchoalveolar lavage.

Treatment

Treatment of HR syndrome is primarily supportive. Management in an intensive care unit is required and the need for mechanical ventilation is common. Therapy with corticosteroids is generally attempted, though their usefulness has not been established as of 2005.

Epidemiology

HR syndrome occurs most frequently among people older than forty years old. It affects men and women equally. There are no known risk factors; in particular, smoking is not associated with increased risk.

Prognosis

Sixty percent of people with HR syndrome will die in the first six months of illness. However, most people who have one episode do not have a second. People who survive often recover lung function completely.

History

HR syndrome was first described in 1935 by Louis Hamman and Arnold Rich.

References

1. ^  HR, AR. Fulminating diffuse interstitial fibrosis of the lungs. Trans Am Clin Climatol Assoc 1935; 51:154.

External links

• synd/3010 at Who Named It
• 00181 at CHORUS
• 1241907254 at GPnotebook
• LUN100 at FPnotebook

•  • [ e] Pathology of respiratory system (, )
Acute upper respiratory infections	Upper respiratory tract infection Common cold Rhinitis Sinusitis Pharyngitis (Strep throat) Tonsillitis Laryngitis Tracheitis Croup Epiglottitis
Influenza and Pneumonia	Influenza Pneumonia (Viral, Bacterial, Bronchopneumonia)
Other acute lower respiratory infections	Bronchitis Bronchiolitis
Other diseases of upper respiratory tract	Vasomotor rhinitis Hay fever Atrophic rhinitis Nasal polyp Adenoid hypertrophy Peritonsillar abscess Vocal fold nodule Laryngospasm
Chronic lower respiratory diseases	Emphysema COPD Asthma Status asthmaticus Bronchiectasis
Lung diseases due to external agents	Pneumoconiosis (Coalworker's pneumoconiosis, Asbestosis, Silicosis, Bauxite fibrosis, Berylliosis, Siderosis) Byssinosis Hypersensitivity pneumonitis (Farmer's lung, Bird fancier's lung)
Other, principally affecting the interstitium	Acute respiratory distress syndrome Pulmonary edema Hamman-Rich syndrome Interstitial lung disease
Suppurative and necrotic conditions of lower respiratory tract	Lung abscess Pleural effusion Empyema
Other	Pneumothorax Hemothorax Hemopneumothorax Mendelson's syndrome Respiratory failure Atelectasis Mediastinal emphysema Mediastinitis
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