Rare Medical News
Disease Profile
Acute interstitial pneumonia
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
Age of onset
Adult
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.
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.
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.
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.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
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.
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.
Not applicable
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
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 | |
| 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 |
0011227 | |
| Elevated |
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 | |
Related diseases
Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.
|
Conditions with similar signs and symptoms from Orphanet
|
|---|
|
The differential diagnosis, histologically and clinically, includes acute exacerbation of pulmonary fibrosis, DAD in patients with collagen vascular diseases, DAD of known cause (ARDS), infection (especially Pneumocystis Jiroveci pneumonia and legionellosis) and drug-induced pneumonitis, as well as hypersensitivity pneumonitis and acute eosinophilic pneumonia(see these terms).
Visit the Orphanet disease page for more information.
|
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
-
Children's Interstitial Lung Disease Foundation
6539 Harrison Ave #1045
Cincinnati, OH 45247
E-mail: [email protected]
Website: https://child-foundation.org/
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.
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
- Talmadge E King, Jr, MD. Acute interstitial pneumonia (Hamman-Rich syndrome). UpToDate. June 2017; Accessed 7/22/2017.
- Leslie Litzky, MD. Pathology of Acute Interstitial Pneumonia. Medscape Reference. March 2016; https://emedicine.medscape.com/article/2078523-overview.