Interstitial lung disease (ILD) associated with amyopathic dermatomyositis (ADM) is a rare and sometimes fatal condition whose clinical features are not well understood. The goal of this study was to clarify the characteristics of ILD based on its development. Eighteen patients diagnosed with ILD associated with ADM were assigned to 1 of 2 groups: (1) a rapidly progressing group, which included patients who developed abnormal lung findings within 1 month of being diagnosed with ADM (n=9); or (2) a slowly progressing group, which including patients who developed lung findings greater than 1 month after diagnosis of ADM (n=9). Serum creatine phosphokinase and C-reactive protein levels were higher in the rapidly progressing group than in the slowly progressing group. Further, arterial pH was higher and PaO(2)/F(I)O(2) was lower in the rapidly progressing group than in the slowly progressing group. On thoracic high-resolution CT, traction bronchiectasis was present in 4 of the 9 rapidly progressing patients but not in any patients of the slowly progressing group. All 9 slowly progressing patients survived with proper treatment, but only 4 of the 9 rapidly progressing patients survived. In ADM, appropriate investigations are likely required for the early diagnosis of ILD. Our data suggest that ILD associated with ADM can be classified into 2 clinical subtypes based on the time course of pulmonary involvement. Patients with rapid progression in respiratory symptoms should undergo intensive treatment as soon as possible to promote favorable outcomes.
There have been no case reports of selective IgM deficiency with concurrent IgG4 deficiency, various dermal symptoms and a bronchial polyp, as demonstrated in our patient.
Worsening gas exchange during exercise and exacerbation in COPD contributes to systemic hypoxemia and restricts the quality of life. However, pulmonary hemodynamics under such conditions are not well understood. We performed right heart catheterization in six patients with severe COPD (%FEV 1 < 50%) during rest, exercise and exacerbation. Pulmonary artery pressure (Ppa) was a little elevated at rest. The Ppa, as pulmonary artery wedge pressure (Pawp) and cardiac index were significantly increased during bicycle ergometer exercise. In contrast, pulmonary vascular resistance significantly increased during exacerbation accompanied by a slightly increased Ppa.Supplemental oxygen resulted in significant decreases in Ppa and Pawp during exercise and Ppa during exacerbation. These findings suggested that the pathological pulmonary hemodynamics are characterized by significant pulmonary hypertension due to dynamic hyperinflation during exercise and a prominent vasoconstrictive reaction under exacerbation. The principal pathophysiology of the pulmonary circulation between exercise and exacerbation might differ in severe COPD. Supplemental oxygen is beneficial in these situations as reflected by improved pathological pulmonary hypertension.2
Idiopathic pulmonary haemosiderosis (IPH) is a diagnosis of exclusion, which is characterized by persistent or recurrent episodes of alveolar haemorrhage. Early diagnosis of IPH, especially in the case of first‐time manifestation, is challenging because previous episodes of alveolar haemorrhage are often difficult to prove. Repeated episodes of alveolar haemorrhage can result in chronic iron‐deficient anaemia and irreversible interstitial fibrosis; thus, early recognition and intervention are desirable in terms of clinical outcome. We report a case of IPH that was diagnosed early by confirming the presence of an increased number of haemosiderin‐laden macrophages with alveolar haemorrhage in repeat bronchoscopy. We wanted to highlight that decreased but sustained attenuation of ground‐glass opacities on high‐resolution computed tomography does not always correlate with successful remission in patients with IPH. Repeat bronchoscopy can be useful in the early recognition of IPH, especially in the case of sustained opacities a few months after alveolar haemorrhage.
Risk factors for seriously ill coronavirus disease 19 (COVID-19) patients have been reported in several studies. However, to date, few studies have reported simple risk assessment tools for distinguishing patients becoming severely ill after initial diagnosis. Hence, this study aimed to develop a simple clinical risk nomogram predicting oxygenation risk in patients with COVID-19 at the first triage. Methods: This retrospective study involved a chart review of the medical records of 84 patients diagnosed with COVID-19 between February 2020 and March 2021 at ten medical facilities. The patients were divided into requiring no oxygen therapy (non-severe group) and requiring oxygen therapy (severe group). Patient characteristics were compared between the two groups. We utilized univariate logistic regression analysis to confirm determinants of high risks of requiring oxygen therapy in patients with moderate COVID-19. Results: Thirty-five patients ware in severe group and forty-nine patients were in non-severe group. In comparison with patients in the non-severe group, patients in the severe group were significantly older with higher body mass index (BMI), and had a history of hypertension and diabetes. Serum blood urea nitrogen (BUN), lactic acid dehydrogenase (LDH), and C-reactive protein (CRP) levels were significantly higher in the severe group. Multivariate analysis showed that older age, higher BMI, and higher BUN levels were significantly associated with oxygen requirements. Conclusions: This study demonstrated that age, BMI, and BUN were independent risk factors in the moderate-tosevere COVID-19 group. Elderly patients with higher BMI and BUN require close monitoring and early treatment initiation.☆ ACE2, angiotensin-converting enzyme 2; AUC, area under the curve; BMI, body mass index; BUN, blood urea nitrogen; CI, confidence interval; COVID-19, coronavirus disease 19; CRP, C-reactive protein; IPPV, invasive positive-pressure ventilation; LDH, lactic acid dehydrogenase; MEWS, Modified Early Warning Score; SpO 2, peripheral oxygen saturation; qCSI, quick COVID-19 Severity Index; qSOFA, Quick Sequential Organ Failure Assessment ROC, receiver operating characteristic; SARS-CoV-2, severe acute respiratory syndrome coronavirus 2; SD, standard deviation.
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