Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist’s point of view

Koo, So-My; Uh, Soo Taek

doi:10.3904/kjim.2016.212

Cited by 29 publications

(24 citation statements)

References 86 publications

(95 reference statements)

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“…Both clinical manifestations, as well as histology of ILD, may vary over a wide range; clinical manifestations can be merely complaint of marginal symptoms or as severe as respiratory failure, similarly, histology can vary from simple inflammation to higher grade fibrosis [27]. Due to the wide ranges of clinical presentation, often with no obvious signs and symptoms and negative pathophysiological examinations, the clinical accuracy is sub-optimal for the diagnosis of ILD associated with CTD [21].…”

Section: Discussionmentioning

confidence: 99%

“…Due to the wide ranges of clinical presentation, often with no obvious signs and symptoms and negative pathophysiological examinations, the clinical accuracy is sub-optimal for the diagnosis of ILD associated with CTD [21]. The most common types of pulmonary manifestations in patients with CTD are PAH, NSIP, UIP, desquamative interstitial pneumonia (DIP), organizing pneumonia (OP), and lymphocytic interstitial pneumonia (LIP) [27]. PAH and NSIP were the most commonly observed pulmonary manifestations in our study; the presence of pulmonary manifestation was found to be associated with SLE, MTCD, scleroderma, overlap syndrome, and dermatomyositis, while, it was found to be lesser associated with RA.…”

Section: Discussionmentioning

confidence: 99%

“…Therapeutic options available for the management are very limited and mostly very toxic [28]. Hence, collaborative efforts are needed in designing a multidisciplinary pathological, radiological, and clinical diagnosis and management approach [7,22,[27][28][29][30].…”

Section: Discussionmentioning

confidence: 99%

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Prevalence and Pattern of Pulmonary Manifestation in Patients with Connective Tissue Disorder

Dahani¹,

Arain²,

Riaz³

et al. 2020

Cureus

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Background A substantial number of deaths and morbidities are caused by pulmonary involvement in connective tissue disorders (CTDs). The majority of patients suffering from CTDs show diverse clinical presentations arising in the lungs, the diaphragm, vasculature, and the pleura during the course of their disease. The clinical course is highly variable ranging from asymptomatic to symptomatic and from reversible to an irreversible stage. The most common occurring pulmonary appearance is the interstitial disease of the lung. In our study, we aim to determine the prevalence and pattern of pulmonary manifestation in patients with CTD presenting at a tertiary care hospital of Karachi, Pakistan. Methods A descriptive cross-sectional study was conducted in the department of Rheumatology Medical Unit II, Jinnah Postgraduate Medical Centre (JPMC), Karachi, Pakistan. All patients having CTD were enrolled. Patients with human immunodeficiency virus (HIV) infection, smokers, chronic obstructive pulmonary disease (COPD), bronchogenic carcinoma and patients with underlying cardiac disease were excluded from the study. Pulmonary manifestations like nonspecific interstitial pneumonia (NSIP), pleural effusion, usual interstitial pneumonia (UIP), interstitial pneumonitis, pulmonary arterial hypertension (PAH), tuberculosis, and pulmonary embolism were observed. In addition to this, treatment history and immunological test findings of these patients were also recorded. Results A total of 290 patients with rheumatologic disorders were included in this study, out of which 90.7% (263) were females and the average age of the entire cohort was 35.13 ± 12.53 years. A majority, 71% (206), of the patients were young (≤ 40 years of age). Rheumatoid arthritis (RA) was the most commonly observed rheumatologic disorder in this cohort which was in 70.3% (204) patients. RA was more prevalent in patients above 40 years of age as compared to young patients (≤ 40 years of age) with a frequency of 81% vs. 66%; p=0.012. Pulmonary involvement was observed in 27.2% of the patients while no pulmonary involvement was noted in 72.8% of patients.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Prevalence and Pattern of Pulmonary Manifestation in Patients with Connective Tissue Disorder

Dahani¹,

Arain²,

Riaz³

et al. 2020

Cureus

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“…IP occurring within the context of CTD is referred to as CTD-associated IP (CTD-IP) [4,5]. Approximately 15% of ILD patients were diagnosed as CTD-ILD after evaluation [6]. IP can be the primary or sole manifestation of CTD [7], leading to di culties in accurate diagnosis at the rst clinical visit.…”

Section: Introductionmentioning

confidence: 99%

Phenotypic Clusters and Survival Analyses in Interstitial Pneumonia with Myositis-Specific Autoantibodies

et al. 2020

Preprint

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Abstract Background: Interstitial pneumonia (IP) is one of the common pulmonary complications of idiopathic inflammatory myopathy (IIM), among which myositis-specific autoantibodies (MSA) are specific for IIM diagnosis and prognosis. However, IP patients with MSA (MSA-IP) have not been well described. The study aimed to explore the phenotypic clusters and prognosis of MSA-IP patients.Methods: A total of 124 MSA-IP patients were prospectively enrolled for analysis. Serum MSA were detected using immunoprecipitation. Radiographic patterns of IP were determined according to the classification of idiopathic IPs. The clusters of MSA-IP patients were identified using cluster analysis. Potential risk factors of acute onset and short-term prognosis were also analyzed. Results: There were four clusters of MSA-IP patients. Cluster 1 patients were elders with chronic onset and usual interstitial pneumonia pattern on CT scan. Cluster 2 patients were all positive for anti-aminoacyl-tRNA antibodies, predominantly females and had frequent respiratory symptoms. Patients of cluster 3 showed multi-system involvements with nonspecific interstitial pneumonia pattern. Patients of cluster 4 had severe respiratory symptoms with anti-MDA5. The patients of cluster 3 (OR 6.682, 95% CI 1.560–28.622, P=0.011) or cluster 4 (OR 6.057, 95% CI 1.715–21.388, P=0.005) were susceptible to acute onset. The patients of cluster 4 were prone to disease progression (HR 2.711, 95% CI 1.128–6.519, P=0.034), which was consistent with the Kaplan–Meier curves.Conclusions: Four distinctive clusters were determined by cluster analysis suggesting the characteristics, serological antibodies and prognosis of MSA-IP.

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“…However, CTD‐CLD is difficult to manage and is limited by few therapeutic options. Current pharmacologic therapy for CTD‐ILD usually consists of corticosteroids and immunosuppressive agents such as azathioprine, cyclophosphamide or mycophenolate mofetil . However, the effects of these medications are less clear, which suggests a demand for new drugs with better efficacy and tolerability.…”

Section: Introductionmentioning

confidence: 99%

Iguratimod ameliorates bleomycin‐induced alveolar inflammation and pulmonary fibrosis in mice by suppressing expression of matrix metalloproteinase‐9

Zhao

Zhou

et al. 2019

Int J of Rheum Dis

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Aim To investigate the potential therapeutic efficacy of iguratimod (IGU) on bleomycin (BLM)‐induced pulmonary fibrosis in mice. Methods A total of 75 C57BL/6 mice were randomly and evenly divided into control group, BLM (5 mg/kg) group, BLM + IGU (90 mg/kg) group, BLM + methylprednisolone (MP, 10 mg/kg) group and BLM + pirfenidone (PF, 100 mg/kg) group. The mice were sacrificed on day 7, 14 and 28. The lung tissue was examined by hematoxylin and eosin staining and Masson staining to evaluate the degree of alveolitis and fibrosis, and serum cytokines were measured. Results Histopathological results showed that IGU attenuated BLM‐induced alveolar inflammation and decreased collagen deposition in lung tissue from day 7 till day 28. Both the pathological alveolitis and fibrosis scores in the drug‐treated groups (IGU group, MP group and PF group) were decreased dramatically compared with the BLM group on day 7, 14 and 28 (P < 0.05). There were no statistical significances among these three groups. Cytokine profile showed that IGU decreased the level of tumor necrosis factor‐α (TNF‐α), interleukin (IL)‐1, IL‐6 and matrix metalloproteinase (MMP)‐9 which were up‐regulated by BLM on day 7, 14 and 28 (P < 0.05). Furthermore, there is a strong correlation between the severity of pulmonary fibrosis and serum MMP‐9 levels. Conclusion IGU can decrease BLM‐induced pulmonary fibrosis, and the anti‐fibrotic effect of IGU is mediated partly via inhibition of MMP‐9, which suggests that IGU could potentially be an effective therapeutic strategy for pulmonary fibrosis.

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Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist’s point of view

Cited by 29 publications

References 86 publications

Prevalence and Pattern of Pulmonary Manifestation in Patients with Connective Tissue Disorder

Prevalence and Pattern of Pulmonary Manifestation in Patients with Connective Tissue Disorder

Phenotypic Clusters and Survival Analyses in Interstitial Pneumonia with Myositis-Specific Autoantibodies

Iguratimod ameliorates bleomycin‐induced alveolar inflammation and pulmonary fibrosis in mice by suppressing expression of matrix metalloproteinase‐9

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