link
Bookmarks
Familial Idiopathic Pulmonary Fibrosis
Carlos S. Restrepo, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
0
3
0

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Idiopathic pulmonary fibrosis (IPF)
                • Familial IPF (FIPF)
                • Usual interstitial pneumonia (UIP)
              • Synonyms

                • Familial pulmonary fibrosis
              • Definitions

                • FIPF is subset of IPF
                • No widely accepted definition
                  • ≥ 2 primary biological family members (parent, sibling, offspring) with IPF
                  • Clinical features compatible with IPF + compatible HRCT findings or histologic evidence of UIP

              IMAGING

              • General Features

                • CT Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    Selected References

                                    1. Kropski JA et al: Genetic evaluation and testing of patients and families with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. ePub, 2016
                                    2. Kropski JA et al: The genetic basis of idiopathic pulmonary fibrosis. Eur Respir J. 45(6):1717-27, 2015
                                    3. Ravaglia C et al: Features and outcome of familial idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 31(1):28-36, 2014
                                    4. Talbert JL et al: Familial Interstitial Pneumonia (FIP). Clin Pulm Med. 21(3):120-127, 2014
                                    5. 25837031García-Sancho C et al: Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis. Respir Med. 105(12):1902-7, 2011
                                    6. Rosas IO et al: Early interstitial lung disease in familial pulmonary fibrosis. Am J Respir Crit Care Med. 176(7):698-705, 2007
                                    7. Lee HL et al: Familial idiopathic pulmonary fibrosis: clinical features and outcome. Chest. 127(6):2034-41, 2005
                                    Related Anatomy
                                    Loading...
                                    Related Differential Diagnoses
                                    Loading...
                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Pathology

                                            • Clinical Issues

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Idiopathic pulmonary fibrosis (IPF)
                                                  • Familial IPF (FIPF)
                                                  • Usual interstitial pneumonia (UIP)
                                                • Synonyms

                                                  • Familial pulmonary fibrosis
                                                • Definitions

                                                  • FIPF is subset of IPF
                                                  • No widely accepted definition
                                                    • ≥ 2 primary biological family members (parent, sibling, offspring) with IPF
                                                    • Clinical features compatible with IPF + compatible HRCT findings or histologic evidence of UIP

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Microscopic Features

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Consider

                                                                      Selected References

                                                                      1. Kropski JA et al: Genetic evaluation and testing of patients and families with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. ePub, 2016
                                                                      2. Kropski JA et al: The genetic basis of idiopathic pulmonary fibrosis. Eur Respir J. 45(6):1717-27, 2015
                                                                      3. Ravaglia C et al: Features and outcome of familial idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis. 31(1):28-36, 2014
                                                                      4. Talbert JL et al: Familial Interstitial Pneumonia (FIP). Clin Pulm Med. 21(3):120-127, 2014
                                                                      5. 25837031García-Sancho C et al: Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis. Respir Med. 105(12):1902-7, 2011
                                                                      6. Rosas IO et al: Early interstitial lung disease in familial pulmonary fibrosis. Am J Respir Crit Care Med. 176(7):698-705, 2007
                                                                      7. Lee HL et al: Familial idiopathic pulmonary fibrosis: clinical features and outcome. Chest. 127(6):2034-41, 2005