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BACKGROUND Interstitial Lung Disease (ILD) is a group of diffuse lung parenchymal diseases characterized by the involvement of pulmonary interstitium. High resolution computed tomography (HRCT) is the cross-sectional imaging modality of choice for the diagnosis and follow up of ILD as it is accurate as well as non-invasive. The present study was done to describe the basic HRCT patterns associated with Interstitial Lung Disease and to correlate the HRCT patterns with clinical data to obtain differential diagnosis of Interstitial Lung Disease (ILD). METHODS 100 patients (41 males and 59 females) with a mean age of 50 years were included in this study during the period from July 2017 to June 2018. These patients were primarily diagnosed as having interstitial lung disease solely on the basis of clinical findings and chest x-ray by the Chest Medicine Department of BMCH and then referred to Radiology Department BMCH for HRCT assessment. RESULTS 74 patients were diagnosed to have interstitial lung disease and 26 patients had airway disease as well. The common frequent findings in interstitial lung disease (ILD) were idiopathic pulmonary fibrosis (UIP) (30%), idiopathic NSIP (21%), connective tissue disease related ILD (15%), extrinsic allergic alveolitis (EAA) (9%), pulmonary lymphangitis carcinomatosis (PLC) (6%) and 4% for each respiratory bronchiolitis associated interstitial lung disease (RB-ILD), bronchiolitis obliterans organizing pneumonia (BOOP), and occupational lung disease. Breathlessness on exertion (93.3%) and dry cough (100%) were the most common presenting symptoms of UIP. The HRCT findings of UIP included reticular shadowing (100%), nodular shadowing (16%), associated ground-glass attenuation in 73.3% and peribronchovascular thickening in 53%. The signs of lung fibrosis were honeycombing in 83.3%, traction bronchiectasis (66%), septal thickening (66%), core pulmonale in 30% and associated lung cancer in 10%. CONCLUSIONS UIP was the most common interstitial lung disease observed in our study. HRCT is the modality of choice in assessing diffuse parenchymal lung disease especially in the aspects of disease activity and earlier detection and characterization of disease.

Background: Although increasing data supports the use of transbronchial lung cryobiopsies (TBLCs) for the diagnosis of diffuse parenchymal lung diseases (DPLDs), its role as an alternative to surgical lung biopsy (SLB) is still under debate. The aim of this study was to assess the benefit of additional SLBs performed in selected patients after TBLCs. Method: We conducted a multicentric Belgian prospective trial in which SLBs were performed after TBLCs when the pathological diagnosis was uncertain or if a nonspecific interstitial pneumonia (NSIP) pattern was observed hypothesizing that SLB could provide additional information and that a co-existent UIP pattern could be missed. Results: Eighty-one patients with TBLCs performed for a DPLD were included in the study between April 2015 and December 2019. A specific histological diagnosis was obtained in 52 patients (64%) whereas no pathological diagnosis following TBLCs was obtained in 13 patients (16%) and a pattern suggestive of a NSIP was observed in 16 patients (20%). Fourteen out of these 29 patients had SLBs after TBLCs. SLBs showed a UIP pattern in 11 (79%), a pattern suggestive of a hypersensitivity pneumonitis in two (14%) and a NSIP pattern in one patient (7%). Among the 16 patients with pathological NSIP following TBLCs, six underwent a SLBs showing a UIP in five and confirming a NSIP in one patient only. A retrospective pathological analysis of patients having both procedures showed a lower diagnostic confidence and agreement among pathologists for TBLCs compared to SLBs. Major factors underlying the added value of SLBs were the bigger size of the sample as well as the subpleural localization of the biopsies. Conclusions: TBLCs are useful in the setting of DPLDs with a good diagnostic yield. However, our study suggests that SLB provides critical additional information in case TBLCs are inconclusive or show a pattern suggestive of a NSIP, questioning the accuracy of TBLC to adequately identify this histological pattern., SCOPUS: ar.j, info:eu-repo/semantics/published

Background Heme oxygenase-1 (HMOX1) catalyzes the metabolism of heme into carbon monoxide, ferrous iron, and biliverdin. Through biliverdin reductase, biliverdin becomes bilirubin. HMOX1-deficiency is a rare autosomal recessive disorder with hallmark features of direct antibody negative hemolytic anemia with normal bilirubin, hyperinflammation and features similar to macrophage activation syndrome. Clinical findings have included asplenia, nephritis, hepatitis, and vasculitis. Pulmonary features and evaluation of the immune response have been limited. Case presentation We present a young boy who presented with chronic respiratory failure due to nonspecific interstitial pneumonia following a chronic history of infection-triggered recurrent hyperinflammatory flares. Episodes included hemolysis without hyperbilirubinemia, immunodeficiency, hepatomegaly with mild transaminitis, asplenia, leukocytosis, thrombocytosis, joint pain and features of macrophage activation with negative autoimmune serologies. Lung biopsy revealed cholesterol granulomas. He was found post-mortem by whole exome sequencing to have a compound heterozygous paternal frame shift a paternal frame shift HMOX1(NM_002133.3):c.262_268delGCCCTGGinsCC (p.Ala88Profs*51) and maternal splice donor HMOX1 (c.636 + 2 T > A) consistent with HMOX1 deficiency. Western blot analysis confirmed lack of HMOX1 protein upon oxidant stimulation of the patient cells. Conclusions Here, we describe a phenotype expansion for HMOX1-deficiency to include not only asplenia and hepatomegaly, but also interstitial lung disease with cholesterol granulomas and inflammatory flares with hemophagocytosis present in the bone marrow.

Interstitial lung disease (ILD) is the most frequent organ involvement (found in nearly half) of myositis patients, but it reveals various clinical courses and therapeutic responsiveness according to clinical and serological subsets. Autoantibodies, as well as imaging and histopathological studies, are useful for the classification of ILD in myositis and provide useful information for predicting prognosis and determining treatment. Antisynthetase antibodies are correlated with chronic and recurrent ILD, whereas anti-CADM-140 (MDA5/IFIH1) antibodies are a marker of acute progressive ILD in clinically amyopathic dermatomyositis. Serum KL-6, SP-D, and ferritin are useful biomarkers for monitoring the activity and severity of ILD. Regarding treatment, glucocorticoids are the first-line drug, but additional immunomodulating drugs are also used in refractory patients. Cyclophosphamide and calcineurin inhibitors (cyclosporine and tacrolimus) appear to be the key drugs in the treatment of refractory myositis-ILD. Rituximab may become another candidate if these drugs are not effective.

Tripe palms [(TP); acral acanthosis nigricans (AN)], is a rare cutaneous syndrome in which the palms develop velvety thickening and rugosity that creates an exaggeration or distortion of dermatoglyphics, resembling boiled tripe. In more than 90% of patients, TP is associated with malignancy, predominantly pulmonary and gastric carcinomas.1 However, in approximately 6% to 10% of patients with TP, no associated malignancy is found.1 We report a case of TP associated only with interstitial lung disease (ILD), specifically idiopathic nonspecific interstitial pneumonia (NSIP), and no associated malignancy.