Can Interstitial Pneumonia as the Sole Presentation of Collagen Vascular Diseases Be Differentiated from Idiopathic Interstitial Pneumonia?

Homma, Yukihiko; Ohtsuka, Yoshinori; Tanimura, Kazunori; Kusaka, Hirotaka; Munakata, Mitsuru; Kawakami, Yoshikazu; Ogasawara, Hideki

doi:10.1159/000196457

Cited by 72 publications

(51 citation statements)

References 2 publications

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“…In this particular group of patients, the initial clinical presentation may be essentially indistinguishable from that of IIP [15,18]. Reporting on 68 patients with IIP followed-up for a period ranging 1-11 yrs, HOMMA et al [18] reported on 13 (19%) patients who subsequently developed a CTD. Initial laboratory findings such as levels of antinuclear antibody (ANA) or rheumatoid factor (RF) did not differ between those who did and did not develop a CTD.…”

Section: Ctd Mimicking Iip: Prevalence and Significance Of Early Diagmentioning

confidence: 92%

“…ILD as the sole PM/DM manifestation ILD can be the presenting manifestation in a significant percentage of patients with PM/DM, with other disease symptoms and signs being minimal or entirely absent [17,18,20,38,44]. In a series of 70 patients with PM/DM and ILD reported by DOUGLAS et al [20], ILD was the sole manifestation in 21 (30%) patients.…”

Section: Polymyositis/dermatomyositismentioning

confidence: 96%

“…By and large, these entities exhibit similar radiological and histological characteristics to their idiopathic counterparts and are thus considered indistinguishable [15,[17][18][19]. Despite similarities in histological and radiological profiles, there are significant differences in survival between the two groups.…”

Section: Ctd Mimicking Iip: Prevalence and Significance Of Early Diagmentioning

confidence: 99%

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Occult connective tissue diseases mimicking idiopathic interstitial pneumonias

Toya²,

2007

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In patients with interstitial lung disease (ILD), the diagnosis of idiopathic interstitial pneumonia is usually made after excluding, among other conditions, connective tissue diseases (CTDs). Although in most patients with a CTD and respiratory symptoms, the systemic nature of the disease is obvious, the ILD-related manifestations in CTDs may often dominate the clinical picture or precede systemic findings and thus mimic idiopathic interstitial pneumonia.With the exception of systemic lupus erythematosus, all CTDs may imitate chronic idiopathic interstitial pneumonias. In this setting, clues to an underlying CTD may be entirely absent or include subtle findings from various systems, including skin, vascular and musculoskeletal system or internal organs. Since nonspecific interstitial pneumonia is a relatively frequent histological pattern in CTDs, biopsy reports of nonspecific interstitial pneumonia should also prompt a search for an underlying CTD.Ultimately, diagnosis of a CTD requires confirmation with immunological testing; interpretation of the various laboratory tests should always be carried out in conjunction with clinical findings.The present article reviews specific clinical aspects of connective tissue disease-related interstitial lung disease that may help differentiate it from idiopathic interstitial pneumonia, especially when interstitial lung disease is the predominant or sole manifestation of an occult connective tissue disease.

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Section: Ctd Mimicking Iip: Prevalence and Significance Of Early Diagmentioning

confidence: 92%

Section: Polymyositis/dermatomyositismentioning

confidence: 96%

Section: Ctd Mimicking Iip: Prevalence and Significance Of Early Diagmentioning

confidence: 99%

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Occult connective tissue diseases mimicking idiopathic interstitial pneumonias

Toya²,

2007

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“…In one series of ILD associated with anti-synthetase antibodies, respiratory manifestations occurred before the onset of muscle or skin disease in 55% of the patients [32]. Long-term follow-up of patients with NSIP may lead to the subsequent diagnosis of CTD in a significant proportion of patients [20,33,34], suggesting that subtle extra-thoracic features of systemic disease present at baseline might have been overlooked. Identification of the CTD may be difficult when the onset of disease is acute or subacute, as often observed in inflammatory idiopathic myopathy (especially associated with anti-melanoma differentiation associated gene (MDA5) antibodies) [35].…”

Section: Ctd Features May Be Missed In the Diagnostic Approach Of Ildmentioning

confidence: 99%

Significance of connective tissue diseases features in pulmonary fibrosis

Cottin

2013

Eur Respir Rev

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Interstitial lung disease (ILD) can occur in any of the connective tissue diseases (CTD) with varying frequency and severity, and an overall long-term prognosis that is less severe than that of idiopathic pulmonary fibrosis (IPF). Because ILD may be the presenting manifestation of CTD and/or the dominant manifestation of CTD, clinical extra-thoracic manifestations should be systematically considered in the diagnostic approach of ILD. When present, autoantibodies strongly contribute to the recognition and classification of the CTD. Patients with clinical extrathoracic manifestations of CTD and/or autoantibodies (especially with a high titer and/or the antibody is considered “highly specific” of an autoimmune condition), but who do not fit with established international CTD criteria may be called undifferentiated CTD or “lung-dominant CTD”. Although it remains to be determined which combination of symptoms and serologic tests best identify the subset of patients with clinically relevant CTD features, available evidence suggests that such patients may have distinct clinical and imaging presentation and may portend a distinct clinical course. However, autoantibodies alone when present in IPF patients do not seem to impact prognosis or management. Referral to a rheumatologist and multidisciplinary discussion may contribute to management of patients with undifferentiated CTD

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“…If presenting before, ILDs may be the only manifestation and precede the extrapulmonary CTD manifestation by several months, sometimes by more than 5 years [5, 6]. Since the radiological and histological characteristics of CTD-ILDs are similar to their idiopathic counterparts [7, 8], autoimmune screening is recommended in all patients with suspected idiopathic interstitial pneumonia (IIP).…”

Section: Introductionmentioning

confidence: 99%

Utility of Anti-DSF70 Antibodies to Predict Connective Tissue Disease in Patients Originally Presenting with Idiopathic Interstitial Pneumonia

et al. 2019

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Background: Anti-DFS70 antibodies, corresponding to the dense fine speckled antinuclear antibody (ANA) pattern in HEp-2 substrates, have been observed in chronic inflammatory conditions, cancer and in healthy individuals but in only a small percentage of patients with connective tissue diseases (CTD). Objectives: The study was aimed to investigate the possible role of Anti-DFS70 antibodies to distinguish CTD associated interstitial lung disease (CTD-ILD) from idiopathic interstitial pneumonia (IIP) and to explore potential correlations between anti-DFS70 antibodies and clinical parameters. Methods: Serum samples were collected from 49 healthy controls (HC), 35 scleroderma-ILD (SSc-ILD) patients as negative controls for anti-DFS70 antibody, and 260 patients with the initial diagnosis IIP including 100 nonspecific interstitial pneumonia (NSIP) and 160 idiopathic pulmonary fibrosis (IPF) patients. ANA pattern was identified by indirect immunofluorescence on HEp-2 cells and anti-DFS70 antibodies were measured in serum by ELISA. Results: Serum anti-DFS70 antibodies were less frequently seen in ILD and SSc-ILD patients compared to HCs. Thirty-seven patients (34 initial idiopathic NSIP and 3 initial IPF patients) developed CTD during 24 months of follow-up, most of them combined with ANA positivity and anti-DFS70 antibody negativity. Anti-DFS70 antibody positivity was not significantly different between CTD-ILD and idiopathic ILD. Conclusions: The frequency of serum anti-DFS70 antibody is markedly decreased in patients with ILDs. Anti-DFS70 antibodies may be useful to predict CTD development in ILD patients.

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Can Interstitial Pneumonia as the Sole Presentation of Collagen Vascular Diseases Be Differentiated from Idiopathic Interstitial Pneumonia?

Cited by 72 publications

References 2 publications

Occult connective tissue diseases mimicking idiopathic interstitial pneumonias

Occult connective tissue diseases mimicking idiopathic interstitial pneumonias

Significance of connective tissue diseases features in pulmonary fibrosis

Utility of Anti-DSF70 Antibodies to Predict Connective Tissue Disease in Patients Originally Presenting with Idiopathic Interstitial Pneumonia

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