scholarly journals ANCAassociated vasculitis in a man with scleroderma and pulmonary fibrosis

2018 ◽  
Vol 13 (3) ◽  
pp. 21-24
Author(s):  
Alyson Wong ◽  
Christine McDonald ◽  
John Thenganatt

Systemic sclerosis, or scleroderma, is a connective tissue disease that causes fibrosis of the skin and potentially internal organs (1). The most common lung findings in those with scleroderma are interstitial lung disease and pulmonary hypertension (1). Here we describe a 58-year-old man with scleroderma, interstitial lung disease and pulmonary arterial hypertension. He presented with atypical pulmonary manifestations and an acute kidney injury caused by a new diagnosis of ANCA-associated vasculitis. 

2020 ◽  
pp. 5-7
Author(s):  
Indranil Banerjee ◽  
CH. Radhika ◽  
R. Srinivasan

Rheumatoid Arthritis is a systemic inflammatory disorder that most commonly affects the joints, causing progressive, symmetric, erosive destruction of cartilage and bone and is usually associated with autoantibody production. Although joint disease is the main presentation, there are a number of extra-articular manifestations including subcutaneous nodule formation, vasculitis, inflammatory eye disease and lung disease. Of these manifestations, lung disease is a major contributor to morbidity and mortality. There are a variety of pulmonary manifestations of rheumatoid arthritis, including pulmonary parenchymal disease (interstitial lung disease) and inflammation of the pleura (pleural thickening and effusions), airways (bronchiectasis) and pulmonary vasculature (pulmonary hypertension). The present article reports five clinical cases in order to elucidate the diversity of the pathophysiological mechanisms that underlie rheumatoid arthritis associated pulmonary diseases. The five manifestations are: interstitial lung disease, pulmonary hypertension, pleural effusion, bronchiectasis and pulmonary tuberculosis (due to prolonged use of pneumotoxic anti-rheumatoid drugs). This case series illustrates the diagnostic challenges that may occur in pleuro-pulmonary disease associated with RA and also the importance of early diagnosis.


2020 ◽  
Vol 13 (1) ◽  
pp. 170-175 ◽  
Author(s):  
Mayuko Nakamura ◽  
Ryoichi Matsunuma ◽  
Kei Yamaguchi ◽  
Ryosuke Hayami ◽  
Michiko Tsuneizumi

Everolimus, an inhibitor of the rapamycin pathway, is administered with the combination of an aromatase inhibitor for the treatment of metastatic estrogen receptor (ER)-positive and human epidermal growth factor receptor 2 (HER2)-negative breast cancers. Interstitial lung disease is a well-known major adverse event associated with everolimus treatment, but it is often difficult to distinguish between interstitial lung disease and Pneumocystis pneumonia, a lung infection. Acute kidney injury is another adverse event that is associated with everolimus use. In this article, we report a case of Pneumocystis pneumonia without respiratory symptoms and acute kidney injury induced by everolimus treatment in a patient with ER-positive and HER2-negative metastatic breast cancer.


Author(s):  
Ryo Teramachi ◽  
Hiroyuki Taniguchi ◽  
Yasuhiro Kondoh ◽  
Tomoki Kimura ◽  
Kensuke Kataoka ◽  
...  

2021 ◽  
Vol 11 (2) ◽  
pp. 235-240
Author(s):  
Houari Aissaoui ◽  
Kinan Drak Alsibai ◽  
Naji Khayath

Anti-MDA5 antibodies-associated amyopathic dermatomyositisis a rare autoimmune disease that involve polyarthritis, cutaneous and pulmonary manifestations. The development of rapidly progressing interstitial lung disease is a life-threatening complication. We report the case of a 45-year-old woman without medical history, who was addressed to the Pulmonary Department for a polyarthritis with dry cough and hypoxemic dyspnea. Initially there was neither cutaneous manifestation nor interstitial lung disease on chest CT scan. After a few days, the patient developed fatal acute respiratory failure with diffuse ground glass opacities. Identification of anti-MDA5 antibodies allowed establishing diagnosis, despite the fact that the first immunological assessment was negative. Corticosteroid bolus of 1 g for three days and immunosuppressive treatment by cyclophosphamide was only initiated at the acute respiratory distress syndrome stage. Given the rapidly unfavorable prognosis of this entity of amyopathic dermatomyositis, the testing for anti-MDA5 antibodies should be recommended in case of progressive pulmonary symptoms associated with joint signs in order to identify this disease at an early stage and to begin rapid and adequate management.


2021 ◽  
Vol 18 ◽  
pp. 147997312199456
Author(s):  
Peining Zhou ◽  
Jing Ma ◽  
Guangfa Wang

Several retrospectivee described the association of interstitial lung disease (ILD) and ANCA-associated vasculitis (AAV). However, the relationship between the ILD and mortality in AAV patients have not been established so far. This study aims to estimate the relevance of AAV-associated-ILD (AAV-ILD) and mortality risk by conducting a systematic review and meta-analysis.A comprehensive systematic review was conducted in accordance with the guidelines of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-analyses). PubMed, Embase.com and the Cochrane Library (Wiley) were searched for original observational studies. Summary estimates were derived with a random-effects model and reported as risk ratio (RR), tested for publication bias and heterogeneity. Ten retrospective cohort studies were included, comprising 526 AAV-ILD patients enrolled from 1974 to 2018. Meta-analysis yielded a pooled RR of 2.90 (95% confidence interval 1.77–4.74) for death among those with AAV-ILD compared to control group. UIP pattern was associated with an even poorer prognosis in comparison to non-UIP pattern (RR 4.36, 95% confidence interval 1.14–16.78). Sensitivity analysis suggested that the meta-RR result was not skewed by a single dominant study. ILD might be associated with a higher mortality risk in AAV patients.


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