Sjögren's syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient
A man in his 70s presents with 12 months of progressive dyspnoea, sicca symptoms and Raynaud's phenomenon. Serological testing and tear duct biopsy confirm Sjögren's syndrome (SS). Bilateral nodular-cystic appearances highly suggestive of lymphoid interstitial pneumonia (LIP) are noted on...
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Veröffentlicht in: | BMJ case reports 2022-06, Vol.15 (6), p.e249747 |
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description | A man in his 70s presents with 12 months of progressive dyspnoea, sicca symptoms and Raynaud's phenomenon. Serological testing and tear duct biopsy confirm Sjögren's syndrome (SS). Bilateral nodular-cystic appearances highly suggestive of lymphoid interstitial pneumonia (LIP) are noted on high-resolution computed tomography (HRCT), supported by a 40% lymphocytosis on bronchoalveolar lavage.Biopsy of a non-characteristic additional pulmonary nodule diagnoses light chain deposition disease (LCDD). Extrapulmonary organ involvement is excluded. Pulmonary function tests are well-preserved, and the patient is kept under active surveillance without requiring immunomodulatory treatment.LIP and LCDD both have a strong association with SS. Identification of these disease associations is crucial as they may result in multiorgan involvement or progression to haematological malignancy. This is the first case published in the literature and highlights that a pragmatic approach to investigations can avoid unnecessary procedures, and that treatment may be guided by symptomology. |
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Serological testing and tear duct biopsy confirm Sjögren's syndrome (SS). Bilateral nodular-cystic appearances highly suggestive of lymphoid interstitial pneumonia (LIP) are noted on high-resolution computed tomography (HRCT), supported by a 40% lymphocytosis on bronchoalveolar lavage.Biopsy of a non-characteristic additional pulmonary nodule diagnoses light chain deposition disease (LCDD). Extrapulmonary organ involvement is excluded. Pulmonary function tests are well-preserved, and the patient is kept under active surveillance without requiring immunomodulatory treatment.LIP and LCDD both have a strong association with SS. Identification of these disease associations is crucial as they may result in multiorgan involvement or progression to haematological malignancy. This is the first case published in the literature and highlights that a pragmatic approach to investigations can avoid unnecessary procedures, and that treatment may be guided by symptomology.</description><identifier>ISSN: 1757-790X</identifier><identifier>EISSN: 1757-790X</identifier><identifier>DOI: 10.1136/bcr-2022-249747</identifier><identifier>PMID: 35750435</identifier><language>eng</language><publisher>England: BMJ Publishing Group LTD</publisher><subject>Amyloidosis ; Antibodies ; Biopsy ; Cardiomyopathy ; Case reports ; Case Reports: Unusual association of diseases/symptoms ; Hematology ; Histology ; Humans ; Kidney diseases ; Light ; Lung Diseases, Interstitial - diagnostic imaging ; Lung Diseases, Interstitial - etiology ; Lymphoma ; Male ; Multiple myeloma ; Multiple Myeloma - complications ; Patients ; Pneumonia ; Respiratory Function Tests ; Sjogren's Syndrome - complications ; Sjogren's Syndrome - diagnosis ; Tomography, X-Ray Computed - adverse effects ; Ultrasonic imaging</subject><ispartof>BMJ case reports, 2022-06, Vol.15 (6), p.e249747</ispartof><rights>BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.</rights><rights>2022 BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.</rights><rights>BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ. 2022</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c310t-5efdd5441365c51fa3b2a437d106b3d384699f35d8ae306f88f0e12a9f246fca3</citedby><cites>FETCH-LOGICAL-c310t-5efdd5441365c51fa3b2a437d106b3d384699f35d8ae306f88f0e12a9f246fca3</cites><orcidid>0000-0002-1873-4665 ; 0000-0002-0882-3955</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC9234793/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC9234793/$$EHTML$$P50$$Gpubmedcentral$$H</linktohtml><link.rule.ids>230,314,723,776,780,881,27903,27904,53770,53772</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/35750435$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Steward, Matthew</creatorcontrib><creatorcontrib>Yu, Johannes H</creatorcontrib><creatorcontrib>Gibbons, Michael A</creatorcontrib><title>Sjögren's syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient</title><title>BMJ case reports</title><addtitle>BMJ Case Rep</addtitle><description>A man in his 70s presents with 12 months of progressive dyspnoea, sicca symptoms and Raynaud's phenomenon. Serological testing and tear duct biopsy confirm Sjögren's syndrome (SS). Bilateral nodular-cystic appearances highly suggestive of lymphoid interstitial pneumonia (LIP) are noted on high-resolution computed tomography (HRCT), supported by a 40% lymphocytosis on bronchoalveolar lavage.Biopsy of a non-characteristic additional pulmonary nodule diagnoses light chain deposition disease (LCDD). Extrapulmonary organ involvement is excluded. Pulmonary function tests are well-preserved, and the patient is kept under active surveillance without requiring immunomodulatory treatment.LIP and LCDD both have a strong association with SS. Identification of these disease associations is crucial as they may result in multiorgan involvement or progression to haematological malignancy. This is the first case published in the literature and highlights that a pragmatic approach to investigations can avoid unnecessary procedures, and that treatment may be guided by symptomology.</description><subject>Amyloidosis</subject><subject>Antibodies</subject><subject>Biopsy</subject><subject>Cardiomyopathy</subject><subject>Case reports</subject><subject>Case Reports: Unusual association of diseases/symptoms</subject><subject>Hematology</subject><subject>Histology</subject><subject>Humans</subject><subject>Kidney diseases</subject><subject>Light</subject><subject>Lung Diseases, Interstitial - diagnostic imaging</subject><subject>Lung Diseases, Interstitial - etiology</subject><subject>Lymphoma</subject><subject>Male</subject><subject>Multiple myeloma</subject><subject>Multiple Myeloma - complications</subject><subject>Patients</subject><subject>Pneumonia</subject><subject>Respiratory Function Tests</subject><subject>Sjogren's Syndrome - complications</subject><subject>Sjogren's Syndrome - diagnosis</subject><subject>Tomography, X-Ray Computed - adverse effects</subject><subject>Ultrasonic imaging</subject><issn>1757-790X</issn><issn>1757-790X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNpdkcuKFDEUhgtRnGGctTsJuNBNOblWqjaCDN5gwIUK7sKpXLrSVCVlkhJ64Wv5Ar6YaXocRrPJIefLn_Pnb5qnBL8ihHVXo04txZS2lA-SywfNOZFCtnLA3x7eq8-ay5z3uC5GeM_Z4-aMCSkwZ-K8-fl5__vXLtnwIqN8CCbFxSLICJCGLVsUHRpjmdB8WNYpeoN8KDbl4ouHGa3BbksMHhAEg2a_mwrSE_iAjF1jrlCspc8WqlQ9BZR92M0WrVC8DeVJ88jBnO3l7X7RfH339sv1h_bm0_uP129uWs0ILq2wzhjBeTUttCAO2EiBM2kI7kZmWM-7YXBMmB4sw53re4ctoTA4yjungV00r0-66zYu1uj6dIJZrckvkA4qglf_doKf1C7-UANlXA6sCry8FUjx-2ZzUYvP2s4zBBu3rGjXk_qhvOsr-vw_dB-3FKq9I4U7KTomKnV1onSKOSfr7oYhWB3TVTVddUxXndKtN57d93DH_82S_QEK1KOJ</recordid><startdate>20220601</startdate><enddate>20220601</enddate><creator>Steward, Matthew</creator><creator>Yu, Johannes H</creator><creator>Gibbons, Michael A</creator><general>BMJ Publishing Group LTD</general><general>BMJ Publishing Group</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7RV</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>BTHHO</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0002-1873-4665</orcidid><orcidid>https://orcid.org/0000-0002-0882-3955</orcidid></search><sort><creationdate>20220601</creationdate><title>Sjögren's syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient</title><author>Steward, Matthew ; Yu, Johannes H ; Gibbons, Michael A</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c310t-5efdd5441365c51fa3b2a437d106b3d384699f35d8ae306f88f0e12a9f246fca3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2022</creationdate><topic>Amyloidosis</topic><topic>Antibodies</topic><topic>Biopsy</topic><topic>Cardiomyopathy</topic><topic>Case reports</topic><topic>Case Reports: Unusual association of diseases/symptoms</topic><topic>Hematology</topic><topic>Histology</topic><topic>Humans</topic><topic>Kidney diseases</topic><topic>Light</topic><topic>Lung Diseases, Interstitial - diagnostic imaging</topic><topic>Lung Diseases, Interstitial - etiology</topic><topic>Lymphoma</topic><topic>Male</topic><topic>Multiple myeloma</topic><topic>Multiple Myeloma - complications</topic><topic>Patients</topic><topic>Pneumonia</topic><topic>Respiratory Function Tests</topic><topic>Sjogren's Syndrome - complications</topic><topic>Sjogren's Syndrome - diagnosis</topic><topic>Tomography, X-Ray Computed - adverse effects</topic><topic>Ultrasonic imaging</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Steward, Matthew</creatorcontrib><creatorcontrib>Yu, Johannes H</creatorcontrib><creatorcontrib>Gibbons, Michael A</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Nursing & Allied Health Database</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>BMJ Journals</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Nursing & Allied Health Premium</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>BMJ case reports</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Steward, Matthew</au><au>Yu, Johannes H</au><au>Gibbons, Michael A</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Sjögren's syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient</atitle><jtitle>BMJ case reports</jtitle><addtitle>BMJ Case Rep</addtitle><date>2022-06-01</date><risdate>2022</risdate><volume>15</volume><issue>6</issue><spage>e249747</spage><pages>e249747-</pages><issn>1757-790X</issn><eissn>1757-790X</eissn><abstract>A man in his 70s presents with 12 months of progressive dyspnoea, sicca symptoms and Raynaud's phenomenon. Serological testing and tear duct biopsy confirm Sjögren's syndrome (SS). Bilateral nodular-cystic appearances highly suggestive of lymphoid interstitial pneumonia (LIP) are noted on high-resolution computed tomography (HRCT), supported by a 40% lymphocytosis on bronchoalveolar lavage.Biopsy of a non-characteristic additional pulmonary nodule diagnoses light chain deposition disease (LCDD). Extrapulmonary organ involvement is excluded. Pulmonary function tests are well-preserved, and the patient is kept under active surveillance without requiring immunomodulatory treatment.LIP and LCDD both have a strong association with SS. Identification of these disease associations is crucial as they may result in multiorgan involvement or progression to haematological malignancy. This is the first case published in the literature and highlights that a pragmatic approach to investigations can avoid unnecessary procedures, and that treatment may be guided by symptomology.</abstract><cop>England</cop><pub>BMJ Publishing Group LTD</pub><pmid>35750435</pmid><doi>10.1136/bcr-2022-249747</doi><orcidid>https://orcid.org/0000-0002-1873-4665</orcidid><orcidid>https://orcid.org/0000-0002-0882-3955</orcidid></addata></record> |
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subjects | Amyloidosis Antibodies Biopsy Cardiomyopathy Case reports Case Reports: Unusual association of diseases/symptoms Hematology Histology Humans Kidney diseases Light Lung Diseases, Interstitial - diagnostic imaging Lung Diseases, Interstitial - etiology Lymphoma Male Multiple myeloma Multiple Myeloma - complications Patients Pneumonia Respiratory Function Tests Sjogren's Syndrome - complications Sjogren's Syndrome - diagnosis Tomography, X-Ray Computed - adverse effects Ultrasonic imaging |
title | Sjögren's syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient |
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