Rapidly progressive fatal interstitial lung disease in a patient with systemic sclerosis

Phillips, Kristine; Byrne-Dugan, Cathryn J.; Batterson, Eric; Seibold, James R.

doi:10.1038/nrrheum.2009.30

Cited by 8 publications

(5 citation statements)

References 16 publications

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“…A diffuse alveolar damage pattern is most frequently seen in the context of RA, PM/DM, SLE or of an undifferentiated CTD, 15 while it is exceedingly rare in the context of SSc, with only a handful of case reports in the literature. 16,17 A diffuse alveolar damage pattern can also occur on a background of a fibrotic ILD, just as acute exacerbation can occur in IPF (DAD occurring on a background of UIP). Finally, with the exception of SSc-ILD, a combination of histological patterns is quite frequent in the context of CTDs (Figure 1), and should in itself raise the possibility of a 'forme fruste' of CTD even in patients who do not yet present with signs and symptoms suggestive of an underlying connective tissue disorder.…”

Section: Histological Patternsmentioning

confidence: 99%

Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?

2011

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The lung is frequently involved in connective tissue diseases (CTDs), although the frequency of lung manifestations varies according to the type of CTD. Interstitial lung diseases (ILD) are frequently seen in CTDs, particularly systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA), accounting for a significant proportion of deaths. A large percentage of patients with CTD-associated ILD has limited and stable disease, not requiring treatment. However, a significant minority has severe and/or progressive disease, necessitating prompt initiation of treatment. CTD-ILD histological patterns include non-specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), diffuse alveolar damage (DAD) and lymphocytic interstitial pneumonia (LIP). NSIP is the most common pattern in all CTDs, except for RA, characterized by a higher frequency of UIP. ILD can present acutely or chronically, with acute presentations being more common in systemic lupus erythematosus and PM/DM. Idiopathic pulmonary fibrosis (IPF) is a progressively worsening ILD characterized by inflammation and fibrosis. The characteristic histological pattern of IPF is UIP. Interestingly, a UIP pattern is associated with a significantly better survival in CTD-related disease compared to the idiopathic variety. Prognosis in IPF is dismal, with a median survival since diagnosis of 2-3 years. No treatment regimen has been shown to improve survival in IPF. By contrast, although there have been only two randomized placebo-controlled trials investigating the effect of immunosuppressive treatment in SSc-associated ILD, clinical experience suggests that immunosuppressive drugs in CTD-related ILDs are capable of benefiting a significant proportion of patients, particularly those with certain histological patterns of disease. This review will essentially focus on CTD-associated ILD and will compare aspects of clinical presentation and management to those of IPF.

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Section: Histological Patternsmentioning

confidence: 99%

Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?

2011

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“…23 In a recent study of CTD-associated DAD, RA accounted for five of nine cases, four of which were in patients with pre-existing RA-ILD, while one was a de novo presentation of the disease. 15 Only single cases of DAD in association with systemic sclerosis (SSc), 15,24,25 Sjogren syndrome (SjS), 26 and mixed connective tissue disease (MCTD) 15,27 have been reported.…”

Section: Patterns Of Lung Injury In Connective Tissue Disease Diffusementioning

confidence: 99%

Pulmonary Pathology in Connective Tissue Disease

Urisman

Jones

2014

Semin Respir Crit Care Med

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A significant proportion of patients with autoimmune connective tissue disease (CTD) show lung involvement that results in clinical interstitial lung disease (ILD). Surgical lung biopsy is helpful for diagnosis of CTD-ILD in many cases. In this review, we discuss the histologic manifestations of different types of CTD-ILD, focusing on patterns of disease and their differential diagnoses. Acquired autoimmune connective tissue diseases will be covered in this review, while lung involvement in vasculitides, heritable connective tissue disorders, and drug-induced CTD-like conditions will not be discussed.

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“…The diagnosis relies on chest high resolution computed tomography (HRCT), pulmonary function testing, Bronchoalveolar lavage (BAL) fluid analysis and lung biopsy. In a case reported by Kristine Phillips, the patient was mainly diagnosed by HRCT, pulmonary function testing and lung biopsy [14]. The characteristics of clinical features are presented as Table 1.…”

Section: Clinical Features and Diagnosismentioning

confidence: 99%

Interstitial Lung Disease in Scleroderma: Clinical Features and Pathogenesis

Luo¹,

Xiao²

2012

Rheumatology

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Interstitial lung disease is a prevalent but worrisome implication in scleroderma. It is now the leading cause of morbidity and mortality since the dramatic reduction in death caused by scleroderma renal crisis. However, the pathogenesis remains unknown. Most observers suggest that lung injury induces microvascular damage and immunologically-mediated inflammation. As oxidative stress and leukotrienes-lipoxins imbalance is observed in-ILD patients, a series of proinflammatory and profibrotic cytokines are also found. Possible biomarkers including SP-D, KL-6, Caveolin-1, IL-6, IL-1 may be useful index for diagnosis and progression, which also suggest the potential mechanism. There are also some studies focusing the relationship between gastroesophageal reflux (GER) and SSc-ILD, which partly explain the mechanism from a new perspective.

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Rapidly progressive fatal interstitial lung disease in a patient with systemic sclerosis

Abstract: Treatment with oxygen, oral and intravenous corticosteroids, mycophenolate mofetil and intravenous cyclophosphamide.

Cited by 8 publications

References 16 publications

Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?

Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?

Pulmonary Pathology in Connective Tissue Disease

Interstitial Lung Disease in Scleroderma: Clinical Features and Pathogenesis

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