Acute Interstitial Pneumonia (AIP)

Relationship to Hamman-Rich Syndrome, Diffuse Alveolar Damage (DAD), and Acute Respiratory Distress Syndrome (ARDS)

Sanjay Mukhopadhyay, M.D.; Joseph G. Parambil, M.D.

Semin Respir Crit Care Med. 2012;33(5):476-485.

Radiology

The main radiological finding in DAD is the presence of bilateral lung infiltrates, which vary from patchy to diffuse and are often described as alveolar. The high-resolution computed tomographic (CT) findings of AIP have been well described.^[20–22,30] They include bilateral ground-glass opacities and/or bilateral airspace consolidation (opacification) (Fig. 1). These findings can be seen in other diseases and are therefore nonspecific. Although traction bronchiectasis and honeycombing have been observed in some patients with putative AIP,^[30] it is likely that these features indicate the presence of an underlying chronic interstitial fibrosing process such as UIP/IPF rather than pure DAD/AIP (see "Acute Exacerbation of IPF").^[20]

(Enlarge Image)

Figure 1.

Bilateral diffuse pulmonary infiltrates in acute interstitial pneumonia (AIP). This radiological picture is not specific for AIP.

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Table 1. Published Series of AIP
First Author (Year)	Number of Patients	Causes of DAD That Were Excluded in the Study	Outcome
Katzenstein (1986)¹²	8	Not specified	6 of 8 died; 2 survived to discharge (one died at 6 months)
Olson (1990)⁹	29	Collagen vascular disease (rheumatoid arthritis, SLE, scleroderma), severe hypotensive episode, infection, COPD, radiation, nitrofurantoin, cyclophosphamide, bleomycin, Wegener granulomatosis, asbestos exposure, hairy cell leukemia	17 of 29 died; 12 survived, some for up to 2 years; no histological features could discriminate survivors from nonsurvivors
Primack (1993)²⁰	9	Infections, including viral Cases with underlying UIP/IPF and SLE were not excluded	8 of 9 died within 3 months of presentation
Ichikado (1997)²¹	14	Not specified	All patients died within 2 weeks to 6 months
Johkoh (1999)²²	36	Not specified	Not available
Vourlekis (2000)²³	13	Infections, cancer chemotherapy, collagen vascular diseases, AIDS, organ transplant, SIRS, toxic exposures	12 of 13 required mechanical ventilation; 4 died in hospital; 8 survived (hospital survival: 67%)
Quefatieh (2003)²⁴	8	Dermatomyositis, infectious pneumonia/sepsis, cocaine, carmustine	7 of 8 survived to hospital discharge
Rice (2003)²⁵	6	Dermatomyositis, rheumatoid arthritis, Still disease, SLE	All patients died (this was an autopsy series)
Bonaccorsi (2003)²⁶	4	Infection, collagen vascular disease	3 of 4 died between 7 and 38 days
Suh (2006)²⁷	10	Infections, drugs, collagen vascular diseases, acute exacerbation of IPF	8 of 10 survived to hospital discharge; survivors were followed from 12 to 78 months; most were asymptomatic on follow-up
Parambil (2007)²⁸	12	Infections, noninfectious complications of transplantation, acute exacerbation of IPF, connective tissue diseases (rheumatoid arthritis, polymyositis/dermatomyositis, diffuse systemic sclerosis, mixed connective tissue disease), drugs, radiation	6 of 12 died (50% hospital mortality)
Avnon (2009)²⁹	9	Cardiac disease, infections, autoimmune disease, malignancy	All patients died within 5–26 days of admission to intensive care unit (100% mortality)

AIDS, acquired immunodeficiency syndrome; COPD, chronic obstructive pulmonary disease; IPF, idiopathic pulmonary fibrosis; SIRS, systemic inflammatory response syndrome; SLE, systemic lupus erythematosus; UIP, usual interstitial pneumonia.

Table 2. Known Causes of Diffuse Alveolar Damage
Causes of DAD	Examples
Infection	Viruses Influenza, seasonal and pandemic^33–35 Herpes simplex virus type 1^28,36 Cytomegalovirus^28,37–39 Adenovirus⁴⁰ Respiratory syncytial virus^28,41 Fungi Disseminated histoplasmosis⁴² Cryptococcal pneumonia²⁸ Nontuberculous mycobacterial infection²⁸
Connective tissue diseases	Rheumatoid arthritis^25,28,43,44 Polymyositis/dermatomyositis^{24,25,28,43,45,46} Systemic lupus erythematosus²⁵ Systemic sclerosis (scleroderma)^28,43,47 Mixed connective tissue disease^28,43 Sjögren syndrome⁴⁸ Anti-Jo-1 tRNA synthetase syndrome⁴⁹
Drugs^50–54	Amiodarone Bleomycin Busulfan Carmustine (BCNU) Cocaine Cyclophosphamide Cytosine-arabinoside (Ara-C) Gemcitabine Nitrofurantoin
Aspiration⁵⁵
Noninfectious complications of organ transplantation²⁸
Oxygen toxicity⁵⁶