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Clinical presentations have been reported as (i) lipoid pneumonias, (ii) acute eosinophilic pneumonias, (iii) pneumonias with pleural effusion, (iv) acute pneumonitis, (v) respiratory bronchiolitis, (vi) interstitial lung disease, (vii) bronchiolitis obliterans organizing pneumonia and (viii) diffuse alveolar hemorrhages [45] (Fig

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In patients with the rare tenascin X-deficient EDS due to mutations in TNXB [28], immunostaining on muscle biopsies shows mildly reduced expression of collagen VI