Hamman-Rich syndrome overview

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hamman-Rich syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Hamman-Rich syndrome is a rare, severe lung disease which usually affects otherwise healthy individuals. HR syndrome is often categorized as both an interstitial lung disease and a form of acute respiratory distress syndrome (ARDS).

Historical Perspective

In 1935, Hamman and rich first described cases with rapidly progressing pulmonary fibrosis of unknown etiology. After that, the eponym, Hamman-Rich syndrome have been used to describe idiopathic pulmonary fibrosis. In 1975, Liebow came up with classification to distinguish between pulmonary fibrosis and idiopathic interstitial lung diseases. In 1986, Katzenstein coined the term acute interstitial pneumonitis. Further studies helped to differentiate acute interstitial pneumonitis from pulmonary fibrosis.

Classification

According to American Thoracic Society/European Respiratory Society (ATS/ERS) 2002 consensus, Acute interstitial pneumonitis is an entity of a group of Idiopathic interstitial lung diseases. The classification is based on clinical, radiological and histopathologic findings. The classification has been updated by ATS/ERS International multidisciplinary panel recently based on the literature review on idiopathic interstitial lung diseases published between 2000-2011.

Pathophysiology

Acute interstitial pneumonitis shows the histopathologic appearance of diffuse alveolar damage. On gross examination, lungs appear firm, heavy and have a dark red or beefy appearance and show irregular areas of consolidation and fibrosis. On microscopic examination, acute interstitial pneumonitis shows bilateral, temporal uniformity of the diffuse alveolar damage, hyaline membrane deposition and extensive fibroblastic and myofibroblastic proliferation.

Causes

There is no specific etiology (idiopathic), that is responsible for developing acute interstitial pneumonitis.

Differentiating [disease name] from other Diseases

Acute interstitial pneumonitis must be differentiated from other diseases that present with respiratory failure and diffuse infiltrates on chest radiographs. Some of the differentials include ARDS, acute eosinophilic pneumonitis, Infections, hypersensitivity pneumonitis, connective tissue diseases, and drug-induced lung toxicity.

Epidemiology and Demographics

  • The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

Acute interstitial pneumonitis occur typically previously healthy individuals in the age group of 50 to 55years.

Gender

Acute interstitial pneumonitis affects men and women equally.

Race

In general there is no racial predilection to acute interstitial pneumonitis.

Risk Factors

Natural History, Complications and Prognosis

  • The majority of patients with [disease name] remain asymptomatic for [duration/years].
  • Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

Symptoms

  • Patients with acute interstitial pneumonitis usually present with flu-like viral illness or upper respiratory tract infection, which progresses very rapidly to acute respiratory failure. Common symptoms include fatigue, headache, myalgia, cough, fever, and dyspnea. The acute onset of symptoms is characteristic of acute interstitial pneumonitis.

Physical Examination

  • Patients with [disease name] usually appear [general appearance].
  • Physical examination may be remarkable for:
  • [finding 1]
  • [finding 2]
  • [finding 3]
  • [finding 4]
  • [finding 5]
  • [finding 6]

Laboratory Findings

  • There are no diagnostic laboratory findings associated with acute interstitial pneumonitis. However, routine laboratory tests may help in identifying alternative diagnoses rather than making a diagnosis of acute interstitial pneumonitis, include abnormal arterial blood gases, physiologic lung testing, complete blood count, and sputum examination, and microbiologic tests.

Imaging Findings

Other Diagnostic Studies

  • Bronchioalveolar lavage and surgical lung biopsy can be helpful in diagnosing other diseases that causing diffuse alveolar damage that present same as acute interstitial pneumonitis.

Treatment

Medical Therapy

  • There is no effective treatment for acute interstitial pneumonitis, Management in general includes supportive therapy and administration of glucocorticosteroids and Immunosuppressive agents

Surgery

Prevention

References

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