Rare Ophthalmology News

Disease Profile

Acute interstitial pneumonia

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
1-9 / 100 000

3,310 - 29,790

US Estimated

1-9 / 100 000

5,135 - 46,215

Europe Estimated

Age of onset

Adult

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ICD-10

J84.1

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Acute interstitial pneumonitis; Hamman-Rich syndrome

Categories

Lung Diseases

Summary

Acute interstitial pneumonia (AIP) is a rare and serious condition that affects the lungs. The signs and symptoms generally develop and progress rapidly. In the early stages of the condition, affected people may experience upper respiratory and/or viral-like symptoms such as cough, shortness of breath, and fever. This is followed by the rapid onset of respiratory failure and the need for mechanical ventilation in the majority of cases. The underlying cause of AIP is unknown. Most cases occur sporadically in people with no family history of the condition. There is, unfortunately, no proven treatment for AIP. Supportive care is generally recommended to address the signs and symptoms of the condition. Glucocorticoid therapy and other immunosuppressive therapies are often used; however, the benefits of these treatments remain unclear.[133607][1]

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Bronchiectasis
Permanent enlargement of the airways of the lungs
0002110
Dyspnea
Trouble breathing
0002094
Ground-glass opacification on pulmonary HRCT
0025179
Hypoxemia
Low blood oxygen level
0012418
Interlobular septal thickening on pulmonary HRCT
0030879
Nodular pattern on pulmonary HRCT
0025392
Peribronchovascular interstitial thickening
0025177
Pulmonary infiltrates
Lung infiltrates
0002113
Respiratory failure
0002878
Reticulonodular pattern on pulmonary HRCT
0025393
30%-79% of people have these symptoms
Crackles
0030830
Cyanosis
Blue discoloration of the skin
0000961
Decreased DLCO
0045051
Fatigue
Tired
Tiredness

[ more ]

0012378
Fever
0001945
Hypertension
0000822
Nonproductive cough
Dry cough
0031246
Pleural effusion
Fluid around lungs
0002202
Tachypnea
Increased respiratory rate or depth of breathing
0002789
5%-29% of people have these symptoms
Arthralgia
Joint pain
0002829
Atelectasis
Partial or complete collapse of part or entire lung
0100750
Chest pain
0100749
Elevated C-reactive protein level
0011227
Elevated erythrocyte sedimentation rate
High ESR
0003565
Elevated serum creatinine
Elevated creatinine
High blood creatinine level
Increased creatinine
Increased serum creatinine

[ more ]

0003259
Lymphadenopathy
Swollen lymph nodes
0002716
Myalgia
Muscle ache
Muscle pain

[ more ]

0003326
Pericardial effusion
Fluid around heart
0001698
Peripheral edema
0012398
Pulmonary fibrosis
0002206
Reduced hematocrit
0031851
Subpleural honeycombing
0031631

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • The Merck Manual provides information on this condition for patients and caregivers.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Merck Manual for health care professionals provides information on Acute interstitial pneumonia.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

        References

        1. Talmadge E King, Jr, MD. Acute interstitial pneumonia (Hamman-Rich syndrome). UpToDate. June 2017; Accessed 7/22/2017.
        2. Leslie Litzky, MD. Pathology of Acute Interstitial Pneumonia. Medscape Reference. March 2016; https://emedicine.medscape.com/article/2078523-overview.