Acute Interstitial Pneumonia

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Retrieved

2021-01-23

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Orphanet

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HMBS, PRSS1, SPINK1, CXCR5, HLA-DRB1, CELA1, FOXP3, TLR4, CFTR, TNF, LTA, LTBR, KRAS, IL33, IFNA13, AMY2A, THY1, IFNA1, TLR2, LILRB1, PDPN, AIP, TGM2, KNTC1, DEGS1, TFPI2, UROD, TP53, MMRN1, ABCF1

HMBS, PRSS1, SPINK1, CXCR5, HLA-DRB1, CELA1, FOXP3, TLR4, CFTR, TNF, LTA, LTBR, KRAS, IL33, IFNA13, AMY2A, THY1, IFNA1, TLR2, LILRB1, PDPN, AIP, TGM2, KNTC1, DEGS1, TFPI2, UROD, TP53, MMRN1, ABCF1, SEC14L2, SMUG1, LOC102724971, LOC102723407, CCR2, MIR181D, MIR193B, MIR34A, MIR150, LOC390714, LINC01193, FCRL3, IL21, KRT20, TLR9, IL22, TGFB1, IGHV3-69-1, IGHV3OR16-7, CD274, PPBP, SST, CXCL8, HSPE1, HLA-E, HLA-A, FECH, ENO1, EGFR, DOCK3, CTLA4, CRP, MS4A1, CALCB, CA2, B2M, ARSA, APOA2, IFN1@, IL10, SKI, IL13, SCT, PPOX, MED1, PLAT, PKD1, PEBP1, NTRK1, NGF, MVD, ATP8, MST1, SMAD4, LAIR1, KCNA3, IDO1, LOC105379528

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A rare rapidly progressive and histologically distinct form of idiopathic interstitial pneumonia.

Epidemiology

Prevalence of Acute interstitial pneumonia (AIP) is estimated at 1 in 25,000.

Clinical description

AIP occurs over a wide age range, with a mean age of approximately 50 years. There is no sex predominance or association with smoking. The onset is acute/subacute (1±3 weeks), with dyspnoea and cough followed by rapid development of respiratory failure and the need for mechanical ventilation in the vast majority of the patients. Fever is present in almost half of the patients on presentation and a history of viral-like symptomatology exists in most cases but extensive investigations for bacterial and viral agents are negative.

Diagnostic methods

The chest radiograph and high-resolution computed tomography (HRCT) scan manifestations of AIP are bilateral and sometimes patchy, and there are alveolar densities associated with areas of ground glass attenuation. Consolidation is seen in the majority of cases, but is not as common as ground glass attenuation. Lung biopsies from patients with AIP show histologic features of the acute and/or organising phases of diffuse alveolar damage (DAD). The exudative phase shows oedema, hyaline membranes, and microvascular thrombi. The organising phase shows loose organising fibrosis, mostly within alveolar septa, and type II pneumocyte hyperplasia. The diagnosis of AIP is made in the appropriate clinical setting in a patient who has a clinical presentation compatible with acute respiratory distress syndrome (ARDS; see this term) but without a clear etiology.

Differential diagnosis

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).

Management and treatment

There is no proven treatment, however high-dose corticosteroids and cyclophosphamide are the drugs usually used.

Prognosis

The prognosis is severe and early mortality is high.