Interstitial Lung Disease in Undifferentiated Forms of Connective Tissue Disease

Fischer, Aryeh; Brown, Kevin K.

doi:10.1002/acr.22394

Cited by 21 publications

(8 citation statements)

References 44 publications

(78 reference statements)

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“…There is growing recognition that ILD might be the first or an isolated manifestation of a CTD. ( 21 ) In 1995, Homma et al, ( 22 ) having followed, for up to 11 years, 68 patients in whom the initial evaluation showed no clinical or serological evidence of CTD, ( 1 ) showed that it is possible for ILD to be the sole presentation of occult CTD. Homma et al ( 22 ) showed that the incidence of definite CTD during long-term follow-up was 19%, similar to the rate observed in the present study, and also found no difference between patients who have definite CTD and LD-CTD patients from a clinical or serological standpoint.…”

Section: Discussionmentioning

confidence: 99%

Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features

et al. 2015

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OBJECTIVE: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). METHODS: This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD). RESULTS: Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. CONCLUSIONS: In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria.

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Section: Discussionmentioning

confidence: 99%

Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features

et al. 2015

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“…All diagnoses were established following the standard criteria. 1 , 9 – 12 ILD–GAP score classes were assigned in accordance with the authors’ original designations. 13 ILD-GAP components are age, gender, forced vital capacity (FVC), and lung diffusion capacity for carbon monoxide (DLco) and type of ILD.…”

Section: Methodsmentioning

confidence: 99%

Auscultation of Velcro Crackles is Associated With Usual Interstitial Pneumonia

et al. 2016

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Auscultation of Velcro crackles has been proposed as a key finding in physical lung examination in patients with interstitial lung diseases (ILDs), especially in idiopathic pulmonary fibrosis (IPF). However, no studies have been carried out to assess the association of Velcro crackles with other clinical variables.We evaluated a cohort of 132 patients, prospectively and consecutively included in our ILD diagnostic program at a tertiary referral center. All patients were auscultated during the physical examination. The patients were divided into 2 groups: “presence” or “nonpresence” of bilateral Velcro crackles.Of all patients assessed, 83 (63%) presented Velcro crackles in the respiratory auscultation. Patients with Velcro crackles usually had more frequently cough and dyspnea at the moment of diagnosis. Forced vital capacity (P = 0.002) and lung diffusion capacity for carbon monoxide (P = 0.04) was lower in these patients. The ILD-GAP index was higher in the group with Velcro crackles (P = 0.01). All patients with usual interstitial pneumonia (UIP) in high-resolution computed tomography and all patients with final IPF diagnosis presented Velcro crackles. In multivariate analysis, the presence of Velcro crackles was independently associated with an UIP pattern.In patients suspected of having ILD, the auscultation of Velcro crackles was associated with UIP, a possibility which must be taken into consideration in early ILD detection in primary care.

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“…CTD may affect many organs, one of which is the respiratory system, including the airways, parenchyma, vascular, pleura and respiratory muscles (1). Interstitial lung disease (ILD) is one of the pulmonary manifestations that may present in CTD, and can increase morbidity and mortality in affected patients (2)(3)(4). In SLE cases, the occurrence of ILD is unusual.…”

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confidence: 99%

Interstitial Lung Disease in Systemic Lupus Erythematosus

Amanda¹,

Fathana²,

Sutoyo³

2019

Respir Case Rep

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Connective tissue disease (CTD) is one of the etiologies of known-cause interstitial lung disease (ILD) that is frequently found with systemic sclerosis, rheumatoid arthritis, mixed-CTD and Sjӧgren syndrome, but which occurs rarely in systemic lupus erythematosus (SLE) patients. A diagnosis of ILD in SLE can be challenging, as the physician should first exclude other pulmonary involvements, including airway disorders, pulmonary infection and vascular abnormality. The management of ILD in SLE and other CTDs patients includes corticosteroid and other immunosuppressive medications. Here we report on two ILD in SLE cases: in one case, ILD was the first sign of SLE; and in another, ILD appeared after the diagnosis of SLE had been established.

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Interstitial Lung Disease in Undifferentiated Forms of Connective Tissue Disease

Cited by 21 publications

References 44 publications

Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features

Lung-dominant connective tissue disease among patients with interstitial lung disease: prevalence, functional stability, and common extrathoracic features

Auscultation of Velcro Crackles is Associated With Usual Interstitial Pneumonia

Interstitial Lung Disease in Systemic Lupus Erythematosus

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