Rapidly progressive fatal interstitial lung disease in a patient with an overlap syndrome of systemic lupus erythematosus and systemic sclerosis

Sacnún, M.; Ferrer, Jaime; Ferrer, Marisol; Pons-Éstel, Bernardo A

doi:10.1016/s2173-5743(11)70010-7

Reumatología Clínica (English Edition)

2011

DOI: 10.1016/s2173-5743(11)70010-7

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Rapidly progressive fatal interstitial lung disease in a patient with an overlap syndrome of systemic lupus erythematosus and systemic sclerosis

M. Sacnún

Jaime Ferrer

Marisol Ferrer

et al.

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Cited by 2 publications

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“…Histologic examination of the lungs revealed UIP. 117 Similarly, a 15-year-old with SLE/PSS overlap syndrome was found to have severe lung fibrosis. 118 A report of tuberculosis in a patient with SLE and DM highlights the importance of infectious complications in overlap syndromes.…”

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confidence: 96%

Pleuropulmonary Pathology in Patients With Rheumatic Disease

Schneider

Gruden

Tazelaar

et al. 2012

Archives of Pathology &Amp; Laboratory Medicine

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Thoracic manifestations of rheumatic disease (RD) are increasingly recognized as a significant cause of morbidity and mortality worldwide. Rheumatologic underpinnings have been identified in a significant proportion of patients with interstitial lung disease. The 5 RDs most frequently associated with pleuropulmonary disease are (1) rheumatoid arthritis, (2) systemic lupus erythematosus, (3) progressive systemic sclerosis, (4) polymyositis/dermatomyositis, and (5) Sjögren syndrome. The onset of thoracic involvement in these diseases is variable. In some patients, it precedes the systemic disease or is its only manifestation. Moreover, there is a wide spectrum of clinical presentation ranging from subclinical abnormalities to acute respiratory failure. Histopathologically, the hallmark features of thoracic involvement by RD are inflammatory, targeting one or more lung compartments. The reactions range from acute to chronic, with remodeling by fibrosis being a common result. Although the inflammatory findings are often nonspecific, certain reactions or anatomic distributions may favor one RD over another, and occasionally, a distinctive histopathology may be present (eg, rheumatoid nodules). Three diagnostic dilemmas are encountered in patients with RD who develop diffuse lung disease: 1) opportunistic infection in the immunocompromised host, 2) drug toxicity related to the medications used to treat the systemic disease, and 3) manifestations of the patient's known systemic disease in lung and pleura. To confidently address the latter, the 5 major RDs are presented here, with their most common pleuropulmonary pathologic manifestations, accompanied by brief clinical and radiologic correlations.

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confidence: 96%

Pleuropulmonary Pathology in Patients With Rheumatic Disease

Schneider

Gruden

Tazelaar

et al. 2012

Archives of Pathology &Amp; Laboratory Medicine

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Fn14, a Downstream Target of the TGF-β Signaling Pathway, Regulates Fibroblast Activation

et al. 2015

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Fibrosis, the hallmark of human injuries and diseases such as serious burns, is characterized by excessive collagen synthesis and myofibroblast accumulation. Transforming growth factor-β (TGF-β), a potent inducer of collagen synthesis, has been implicated in fibrosis in animals. In addition to TGF-β, fibroblast growth factor-inducible molecule 14 (Fn14) has been reported to play an important role in fibrotic diseases, such as cardiac fibrosis. However, the function and detailed regulatory mechanism of Fn14 in fibrosis are unclear. Here, we investigated the effect of Fn14 on the activation of human dermal fibroblasts. In normal dermal fibroblasts, TGF-β signaling increased collagen production and Fn14 expression. Furthermore, Fn14 siRNA blocked extracellular matrix gene expression; even when TGF-β signaling was activated by TGF-β1, fibroblast activation remained blocked in the presence of Fn14 siRNA. Overexpressing Fn14 increased extracellular matrix gene expression. In determining the molecular regulatory mechanism, we discovered that SMAD4, an important TGF-β signaling co-mediator, bound to the Fn14 promoter and activated Fn14 transcription. Taken together, these results indicate that the TGF-β signaling pathway activates Fn14 expression through the transcription factor SMAD4 and that activated Fn14 expression increases extracellular matrix synthesis and fibroblast activation. Therefore, Fn14 may represent a promising approach to preventing the excessive accumulation of collagen or ECM in skin fibrosis.

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Rapidly progressive fatal interstitial lung disease in a patient with an overlap syndrome of systemic lupus erythematosus and systemic sclerosis

Cited by 2 publications

References 24 publications

Pleuropulmonary Pathology in Patients With Rheumatic Disease

Pleuropulmonary Pathology in Patients With Rheumatic Disease

Fn14, a Downstream Target of the TGF-β Signaling Pathway, Regulates Fibroblast Activation

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