(1) The updated classification described 2 rare IIPs: lymphoid interstitial pneumonia (LIP) and pleuroparenchymal fibroelastosis
(PPFE) is an idiopathic interstitial pneumonitis.
(PPFE) was initially described as upper lobe pulmonary fibrosis of unknown etiology by Amitani et al.
Echocardiography is usually the initial diagnostic tool; when the echocardiographer found the imaging feature of the dilation and dysfunction of left ventricular with severe mitral regurgitation, abnormal flow-pattern in the ventricular septum and dilatation of the right coronary artery, and the wide enhancement and thickening of the endocardium, the echocardiographer should not only pay attention to idiopathic endocardial fibroelastosis
(EFE)  and/or idiopathic dilated cardiomyopathy (DCM)  but also should check the origination of the coronary arteries to avoid being misdiagnosed.
More than half of CHB cases are associated with autoimmune and CHB remains a severe life-threatening disorder.,,,,,,,, Autoimmune-associated CHB occurs in 2-5% pregnancies with positive anti-Ro/SSA (the most common one) and La/SSB antibodies, and it has a recurrence rate of 12-25% in a subsequent pregnancy.,,, The perinatal mortality of neonate with CHB researches to 30%, and even higher in the presence of endocardial fibroelastosis
(EFE) and/or dilated cardiomyopathy (DCM)., Previous studies revealed that 64-70% CHB survivors require permanent pacemaker implantation.,,,,,,
Exclusion criteria included also coagulopathy (platelet count < 100000/[mm.sup.3], prothrombin time international normalized ratio-INR > 1.5, and activated partial thromboplastin time-APTT > 35), hypoxemia (Pa[O.sub.2] < 55 mmHg on room air), or hypercapnia (PaC[O.sub.2] > 45 mmHg), severe underlying cardiac disease, and suspicion of pleuroparenchymal fibroelastosis
based on the HRCT aspect (presence of a dense pleural and subpleural consolidation with a reticular pattern, predominantly in the upper lobes) .
The mechanical abnormalities include cardiomyopathy, congestive heart failure, endomyocardial fibroelastosis
, and valvular lesions.
Oyama et al., "Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis
(IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF)," BMC Pulmonary Medicine, vol.
According to the American Thoracic Society and European Respiratory Society, the idiopathic interstitial pneumonias are classified today into: (1) chronic fibrosing idiopathic interstitial pneumonias (idiopathic pulmonary fibrosis and idiopathic nonspecific interstitial pneumonia); (2) smoking-related idiopathic interstitial pneumonias (respiratory bronchiolitis-associated interstitial lung disease and desquamative interstitial pneumonia); (3) acute or subacute idiopathic interstitial pneumonias (cryptogenic organizing pneumonia and acute interstitial pneumonia) and (4) rare idiopathic interstitial pneumonias (lymphoid interstitial pneumonia and idiopathic pleuroparenchymal fibroelastosis
There are several types of primary cardiomyopathies: arrhythmogenic right ventricular dysplasia, dilated (congestive) CMP, hypertrophic CMP, specific CMP (metabolic, inflammatory, ischemic, cirrhotic, etc.), and unclassified CMP (fibroelastosis
, noncompaction cardiomyopathy (spongiform cardiomyopathy), mitochondriopathies, etc.).
Abbreviations CVID: Common variable immunodeficiency DC: Dyskeratosis congenita GLILD: Granulomatous-lymphocytic interstitial lung disease HP1y: Heterochromatin protein 1 gamma IPF: Idiopathic pulmonary fibrosis PPFE: Pleuroparenchymal fibroelastosis
TINF2: TRFFl-Interacting Nuclear Factor 2 UIP: Usual interstitial pneumonia.