An Unusual Case of Interstitial Lung Disease as the Primary Presentation of Mixed Connective Tissue Disease in a Child with Sickle Cell Disease

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An Unusual Case of Interstitial Lung Disease as the Primary Presentation of Mixed Connective Tissue Disease in a Child with Sickle Cell Disease. / Diaz-Gil, Daniel; Popatia, Rizwana.

in: PEDIAT ALLER IMM PUL, Jahrgang 31, Nr. 3, 09.2018, S. 191-193.

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@article{3fff63d8ae57482d9fed9dd1f83ec59c,
title = "An Unusual Case of Interstitial Lung Disease as the Primary Presentation of Mixed Connective Tissue Disease in a Child with Sickle Cell Disease",
abstract = "Mixed connective tissue disease (MCTD) is an autoimmune disease associated with pulmonary manifestations. We report a case of a 15-year-old asymptomatic African American boy with sickle cell disease (SCD), diagnosed with MCTD-related interstitial lung disease as the primary diagnosis after a work-up for worsening pulmonary function tests (PFTs). To our knowledge, this is the first case of MCTD with primary pulmonary manifestations in a pediatric patient with sickle cell anemia with no respiratory symptoms, diagnosed with PFTs, serology, and biopsy. This case emphasizes the need of early pulmonary specialist involvement in SCD and autoimmune disease patients, and that periodic PFTs remain a very useful tool for early diagnosis and disease progression assessment.",
keywords = "autoimmune disease, child, interstitial lung disease, mixed connective tissue disease, pulmonary function testing, sickle cell disease",
author = "Daniel Diaz-Gil and Rizwana Popatia",
note = "Publisher Copyright: {\textcopyright} Copyright 2018, Mary Ann Liebert, Inc., publishers 2018.",
year = "2018",
month = sep,
doi = "10.1089/ped.2017.0851",
language = "English",
volume = "31",
pages = "191--193",
journal = "PEDIAT ALLER IMM PUL",
issn = "2151-321X",
publisher = "Mary Ann Liebert Inc.",
number = "3",

}

RIS

TY - JOUR

T1 - An Unusual Case of Interstitial Lung Disease as the Primary Presentation of Mixed Connective Tissue Disease in a Child with Sickle Cell Disease

AU - Diaz-Gil, Daniel

AU - Popatia, Rizwana

N1 - Publisher Copyright: © Copyright 2018, Mary Ann Liebert, Inc., publishers 2018.

PY - 2018/9

Y1 - 2018/9

N2 - Mixed connective tissue disease (MCTD) is an autoimmune disease associated with pulmonary manifestations. We report a case of a 15-year-old asymptomatic African American boy with sickle cell disease (SCD), diagnosed with MCTD-related interstitial lung disease as the primary diagnosis after a work-up for worsening pulmonary function tests (PFTs). To our knowledge, this is the first case of MCTD with primary pulmonary manifestations in a pediatric patient with sickle cell anemia with no respiratory symptoms, diagnosed with PFTs, serology, and biopsy. This case emphasizes the need of early pulmonary specialist involvement in SCD and autoimmune disease patients, and that periodic PFTs remain a very useful tool for early diagnosis and disease progression assessment.

AB - Mixed connective tissue disease (MCTD) is an autoimmune disease associated with pulmonary manifestations. We report a case of a 15-year-old asymptomatic African American boy with sickle cell disease (SCD), diagnosed with MCTD-related interstitial lung disease as the primary diagnosis after a work-up for worsening pulmonary function tests (PFTs). To our knowledge, this is the first case of MCTD with primary pulmonary manifestations in a pediatric patient with sickle cell anemia with no respiratory symptoms, diagnosed with PFTs, serology, and biopsy. This case emphasizes the need of early pulmonary specialist involvement in SCD and autoimmune disease patients, and that periodic PFTs remain a very useful tool for early diagnosis and disease progression assessment.

KW - autoimmune disease

KW - child

KW - interstitial lung disease

KW - mixed connective tissue disease

KW - pulmonary function testing

KW - sickle cell disease

UR - http://www.scopus.com/inward/record.url?scp=85053721229&partnerID=8YFLogxK

U2 - 10.1089/ped.2017.0851

DO - 10.1089/ped.2017.0851

M3 - SCORING: Journal article

AN - SCOPUS:85053721229

VL - 31

SP - 191

EP - 193

JO - PEDIAT ALLER IMM PUL

JF - PEDIAT ALLER IMM PUL

SN - 2151-321X

IS - 3

ER -