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Rare forms of interstitial lung disease
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
Diffuse panbronchiolitis is an unusual form of bronchiolitis of unknown cause described mainly in Asia, particularly in Japanese adults, and is characterized by diffuse bronchiolar inflammation and chronic sinusitis (82,83). Few cases occurring in non-Asian patients have been described in the United States and Europe (84,85). Patients are typically middle aged and present with sub-acute onset of cough productive of purulent sputum, dyspnoea and evidence of airflow obstruction.
Chronic lung allograft dysfunction other than bronchiolitis obliterans syndrome
Published in Wickii T. Vigneswaran, Edward R. Garrity, John A. Odell, LUNG Transplantation, 2016
Stijn E. Verleden, Robin Vos, Bart M. Vanaudenaerde, Geert M. Verleden
However, the introduction of azithromycin caused a paradigm shift. Azithromycin is a neomacrolide antibiotic (also called an azalide) with a 15-membered macrolactone ring structure that is derived from Streptomyces species.14 It was first introduced into clinical practice to treat diffuse panbronchiolitis, for which it was used mainly because of its effect against Pseudomonas.15 Later studies were able to show that azithromycin was beneficial in patients who suffered from cystic fibrosis and were chronically colonized with Pseudomonas.16 At approximately the same time Gerhardt and colleagues published the first report demonstrating a 0.5-L improvement in FEV1 in five of six patients with BOS who were treated with azithromycin (250 mg three times per week).17 This pilot study was followed by several other smaller studies that all confirmed improvement in FEV1 in a subset of patients with BOS.18,19 The different studies have been summarized in a comprehensive review.14 Administration of azithromycin to all 412 patients with BOS in all the retrospective studies led to an improvement in FEV1 in 145 (35%) of them (mean increase, 7.6%). A representative example is presented in Figure 34.1. However, not all the studies have reported beneficial effects: in particular, one study failed to show any improvement in FEV1 but did at least show stabilization of FEV1, which is also of great value in patients with BOS.20 Not all centers use azithromycin; instead, some use clarithromycin, another macrolide with comparable anti-inflammatory effects.21 However, clarithromycin should be used with caution because of its known effect on trough levels of calcineurin,22 and its possible side effects, though limited, should also be borne in mind. Azithromycin remains an antibiotic that can cause bacterial resistance. Also, a slight increase in risk for cardiovascular death during treatment with azithromycin for 5 days has been noted23; in a young cohort, however, long-term treatment did not show any adverse cardiovascular events associated with macrolide treatment.24 Rare reports of prolongation of the QT interval in patients undergoing azithromycin therapy do exist, but such risk appears to increase only in vulnerable patients.25 The same statement holds true for cardiac arrhythmias: azithromycin does not generally increase the risk for arrhythmias, although extra caution should be exercised when azithromycin is used in patients with additional risk factors.26 Furthermore, it seems that BAL neutrophilia can redevelop despite azithromycin therapy, in which case it may be associated with a renewed decline in FEV1 similar to that with BOS but refractory to treatment. The prevalence and the significance of these findings remain to be established.
Clinical manifestations and long-term prognosis of Good syndrome: Results from a single-center cohort study from China
Published in Modern Rheumatology, 2021
Yueting Li, Zhuoran Yao, Mengdi Jiang, Yuxue Nie, Ruxuan Chen, Naixin Liang, Huaxia Yang, Xuan Zhang, Fengchun Zhang
The systemic involvements rather than autoimmune manifestations in GS are complicated. Bronchiectasis occurred in 38–45% of patients with GS [5,10], and the prevalence was related to the histologic type of thymoma [10]. Diffuse panbronchiolitis, as an inflammatory condition of the bronchioles due a lack of immune system resistance, was reported to tend to present in patients with thymoma [22]. Diarrhea was present in 3–32% of patients with GS, and specific pathogens could only be identified in a few patients [9,10]. Colitis and duodenal inflammation in patient with GS responded to prednisone or sulfasalazine, indicating a potential pathogenesis of autoimmune imbalance [23,24]. One patient in our cohort with chronic noninfectious diarrhea responded transiently to IVIG but had a long-term remission after receiving the intravenous mesenchymal stem cell treatment. Another case reported the improvement of noninfectious diarrhea in a patient with Good's syndrome by fecal microbiota transplantation [25]. More evidence is needed to prove the effectiveness and safety of these novel treatments of GS. Compared to the previous report [9], the positivity of ANAs in this cohort (15%) was lower and connective tissue diseases such as systemic lupus erythematous and Sjögren’s syndrome were not detected in our cohort. These discrepancies might be due to ethnicity differences of recruited patients.
HTLV-I associated bronchioloalveolar disorder (HABA): disease concept and differential diagnosis of an unsolved disease entity
Published in Expert Review of Anti-infective Therapy, 2023
Akihiro Ohmoto, Shigeo Fuji, Satoshi Kohmo, Kaoruko Katsura
HABA is thought to develop as a result of an immune reaction in the lungs by activated T-cells, which are induced by HTLV-1 [11]. This was demonstrated in previous research, such as the study by Kadota et al. who compared the clinical characteristics of 15 patients with HTLV-1-associated bronchiolitis and 43 with diffuse panbronchiolitis. Although the clinical and radiological features were similar between the two study groups, the percentage of interleukin-2 receptor (IL-2R)-positive cells in the bronchoalveolar lavage fluid (BALF) was higher in the HTLV-1 carriers. Moreover, elevated IL-2R levels in HTLV-1 carriers can be explained by T-cell activation [12]. The onset mechanism is chiefly explained by the activation of the Tax protein and the production of pro-inflammatory cytokines (e.g. interferon-γ) or chemokines (e.g. macrophage inflammatory protein-1 alpha (MIP-1α)) [13]. From BALF sample analysis, Seki et al. correlated elevated MIP-1α levels with a higher percentage of CD3+/CD25+ lymphocytes suggestive of HTLV-1 infection [14]. Furthermore, Nakayama et al. reported that the mRNA expression levels of HTLV-I bZIP factor (HBZ) and forkhead box P3 (Foxp3) in BALF samples were higher in HTLV-1 infected patients with lung lesions than in healthy cohorts [15]. In addition, Yamamoto et al. analyzed BALF samples from HTLV-1 infected and uninfected patients with diffuse pan-bronchiolitis and reported that the CD3+/CD25+ lymphocyte percentage and MIP-1α and IP-10 levels were higher in the HTLV-1 infected cases [16]. Their study also showed a positive correlation between the CD3+/CD25+ lymphocyte percentage and MIP-1α or IP-10 levels. In summary, elevated chemokine levels, such as MIP-1α are associated with increased HTLV-1 infected cells in BALF samples, and may suggest the presence of HABA. It should be noted that we do not know whether any mechanistic difference exists between ATL and non-neoplastic HAM/TSP or HABA.