Arterial and interstitial remodelling processes in non‐specific interstitial pneumonia: systemic sclerosis versus idiopathic

Carvalho, Erika Franco de; Parra, Edwin Roger; Souza, R De; Saber, Alexandre M Ab'; Machado, Juliana; Capelozzi, Vera Luíza

doi:10.1111/j.1365-2559.2008.03072.x

Cited by 14 publications

(14 citation statements)

References 37 publications

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“…[7] Other reports regarding PFT abnormalities in patients with both idiopathic and nonidiopathic forms of NSIP have also demonstrated similar reductions in TLC and DLCO, though no correlation with mPAP was made. [43–45] The degree of pulmonary hypertension in our patients was not predicted by the degree of restriction on their PFTs, though a severe reduction in DLCO may have served as a clue to the presence of pulmonary vascular disease.…”

Section: Discussionmentioning

confidence: 77%

Severe Pulmonary Hypertension in Idiopathic Nonspecific Interstitial Pneumonia

et al. 2012

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Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD), particularly in idiopathic pulmonary fibrosis (IPF) and ILD associated with connective tissue disease, where the underlying pathology is often a nonspecific interstitial pneumonia (NSIP) pattern. The degree of PH in ILD is typically mild to moderate and radiographic changes of ILD are usually prominent. We describe four patients with idiopathic NSIP and severe PH (mPAP > 40 mmHg). The average mean pulmonary artery pressure was 51±7 mmHg and pulmonary vascular resistance was 13±4 Wood's units. Pulmonary function was characterized by mild restriction (total lung capacity 63–94% predicted) and profound reductions in DLCO (19–53% predicted). Computed tomographic imaging revealed minimal to moderate interstitial thickening without honeycombing. In two of the cases, an initial clinical diagnosis of idiopathic pulmonary arterial hypertension was made. Both were treated with intravenous epoprostenol, which was associated with worsening of hypoxemia. All four patients died or underwent lung transplant within 4 years of PH diagnonsis. Lung pathology in all four demonstrated fibrotic NSIP with medial thickening of the small and medium pulmonary arteries, and proliferative intimal lesions that stained negative for endothelial markers (CD31 and CD34) and positive for smooth muscle actin. There were no plexiform lesions. Severe pulmonary hypertension can therefore occur in idiopathic NSIP, even in the absence of advanced radiographic changes. Clinicians should suspect underlying ILD as the basis for PH when DLCO is severely reduced or gas exchange deteriorates with pulmonary vasodilator therapy.

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Section: Discussionmentioning

confidence: 77%

Severe Pulmonary Hypertension in Idiopathic Nonspecific Interstitial Pneumonia

et al. 2012

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“…However, immunohistochemical analysis could not be performed in our analysis. Moreover, vasculopathy and fibrosis in SSc appear to be different pathogenetic processes [ 6 ], determination of vasculopathy's role in accelerating emphysema is required in the future.…”

Section: Discussionmentioning

confidence: 99%

Emphysema formation in a never-smoker with scleroderma-related interstitial pneumonia

Yamakawa

Takemura

Baba

et al. 2018

Respiratory Medicine Case Reports

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“…Molecular biology is rarely necessary to identify a lymphoid neoplasm, viral-associated infections [HIV, human herpesvirus-8 (HHV8), Epstein-Barr virus (EBV)], or Pneumocystis jiroveci cysts. 4 NSIP morphology in nonidiopathic cases presents with some variation or additional elementary lesions: pleuritis and follicular hyperplasia is more evident in CTD 17 ; a combination of different patterns (NSIP associated with patchy DAD or organizing pneumonia) are more characteristically observed in polymyositis/dermatomyositis, NSIP associated with vascular changes (increase of collagen and elastic fibers) in peripheral pulmonary arteries and arterioles is typically observed in scleroderma, 18,19 whereas epithelial damage (up and down pattern), bronchiolitis, and lymphoid infiltration of the bronchiolar epithelium are typically observed in GVHDrelated pneumonitis. 20 Finally, achieving interobserver agreement among pathologists about NSIP pattern in idiopathic cases is challenging, 21,22 and quantitative criteria and more specific markers are still lacking.…”

Section: Nsip As a Histopathologic Patternmentioning

confidence: 99%

“…Systemic symptoms (e.g., arthralgias, fever, Raynaud phenomenon) and serological abnormalities (e.g., elevated erythrocyte sedimentation rate, antinuclear antibodies, or rheumatoid factor) have been reported in patients with IIP, suggesting an autoimmune background. 12,[37][38][39][40] Furthermore, the NSIP histological pattern is the most common pattern in patients with CTD 18,19,41 [e.g., undifferentiated CTD (UCTD), polymyositis/dermatomyositis, scleroderma, Sjögren syndrome, and rarely, rheumatoid arthritis]. 42 The hypothesis that NSIP might represent the first clinical presentation of various CTDs was raised by Sato et al, 43 who retrospectively reviewed six patients with histologically proven NSIP who later developed typical CTD, more than 6 months after the first presentation of NSIP.…”

Section: Nsip and Autoimmune Disordersmentioning

confidence: 99%

Current Status of Idiopathic Nonspecific Interstitial Pneumonia

Poletti¹,

Romagnoli

Piciucchi

et al. 2012

Semin Respir Crit Care Med

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Pulmonary pathologists were aware of cases of idiopathic interstitial pneumonia (IIP) that morphologically did not fit Liebow's classification scheme. These cases were labeled as "cellular interstitial pneumonia" or "chronic interstitial pneumonia not otherwise specified." The term nonspecific interstitial pneumonia (NSIP) was first used in relation to a pattern of lung interstitial inflammation seen in association with human immunodeficiency virus (HIV) infection. In 1994 NSIP was used to indicate a group of subacute or chronic interstitial pneumonias characterized morphologically by interstitial inflammation or fibrosis or both, with preservation of the lung architecture and the absence of typical findings for any of the other main categories of IIP (mainly usual interstitial pneumonia, desquamative interstitial pneumonia, and bronchiolitis obliterans organizing pneumonia). Although these patients presented with "nonspecific" lung histology (categorized as cellular and fibrotic variants), and with a broad spectrum of associated clinical conditions, such as connective tissue diseases (CTDs), environmental exposure, and previous acute lung injury, they showed some peculiar clinical aspects, including favorable response to corticosteroid treatment and overall good prognosis.The clinical and radiographic profiles were better defined in the last decade. The NSIP pattern is the histological background of a subacute/chronic interstitial pneumonitis that may be observed in many conditions, including CTD, drug-induced lung disease, hypersensitivity pneumonitis, slowly healing diffuse alveolar damage (DAD), relapsing organizing pneumonia, occupational exposure, immunodeficiency (mainly HIV infection), graft versus host disease (GVHD), familial pulmonary fibrosis, immunoglobulin G4 (IgG4)-related sclerosing disease, with or without overlap features with Rosai-Dorfman disease, multicentric Castleman disease, and myelodysplastic syndrome. Rarely, NSIP is the histology recognized in patients with idiopathic interstitial pneumonitis, in whom efforts to find potential causative exposures are futile. This entity occurs mostly in middle-aged, never-smoker women, with a likely association with an autoimmune background. High-resolution computed tomographic (HRCT) scans typically demonstrate ground-glass attenuation with a bibasilar distribution, or in the fibrotic variant, ground-glass attenuation along with reticular lines and traction bronchiectasis. The prognosis is good compared with idiopathic pulmonary fibrosis (IPF), and therapeutic options include mainly corticosteroids and immunosuppressive agents. Recently a more precise definition of clinical profiles and radiographic findings of idiopathic NSIP allows consideration of less invasive diagnostic procedures (bronchoalveolar lavage, transbronchial lung biopsy). Better understanding of pathogenetic mechanisms might widen the therapeutic horizon giving a role to new therapeutic options in more severe cases.

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Arterial and interstitial remodelling processes in non‐specific interstitial pneumonia: systemic sclerosis versus idiopathic

Cited by 14 publications

References 37 publications

Severe Pulmonary Hypertension in Idiopathic Nonspecific Interstitial Pneumonia

Severe Pulmonary Hypertension in Idiopathic Nonspecific Interstitial Pneumonia

Emphysema formation in a never-smoker with scleroderma-related interstitial pneumonia

Current Status of Idiopathic Nonspecific Interstitial Pneumonia

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