PMID- 36837534
OWN - NLM
STAT- MEDLINE
DCOM- 20230228
LR  - 20230228
IS  - 1648-9144 (Electronic)
IS  - 1010-660X (Print)
IS  - 1010-660X (Linking)
VI  - 59
IP  - 2
DP  - 2023 Feb 10
TI  - Recurrent Exacerbations and Evolution into Polymyositis in a Patient with 
      Interstitial Pneumonia with Autoimmune Features: A Case Report and Literature 
      Review.
LID - 10.3390/medicina59020330 [doi]
LID - 330
AB  - Interstitial pneumonia with autoimmune features (IPAF) is a new disease entity 
      proposed in 2015. Numerous questions regarding IPAF require clarification, 
      including diagnostic criteria, standard managements for stable disease and 
      exacerbation, and prognosis. We report a case of a 67-year-old Asian woman who 
      presented with progressive dyspnea. Chest computed tomography (CT) scans revealed 
      nonspecific interstitial pneumonia. Serologic testing indicated positive 
      anti-Jo-1 without presence of extrathoracic manifestations. An IPAF diagnosis was 
      made after a multidisciplinary discussion. The patient experienced a severe 
      exacerbation requiring mechanical ventilation, and she was successfully salvaged 
      with methylprednisolone pulse therapy and single-dose cyclophosphamide. During 
      the one-year follow-up, she reported bilateral leg muscle weakness with 
      noticeably elevated serum creatine kinase, suggesting polymyositis. The 
      development of malignancy was also noted 15 months after the initial 
      presentation, and the patient eventually died. This report demonstrated 
      successful salvage treatment with glucocorticoid pulse therapy for IPAF with 
      acute exacerbation. However, the maintenance therapy failed to control disease 
      progression. The treatment strategies for exacerbation and stable disease in IPAF 
      remain unknown and need further studies. Given the high risk of evolution into a 
      defined connective tissue disease (CTD), regular evaluation of the clinical 
      features and biomarkers of CTDs is essential for patients with IPAF.
FAU - Huang, Chien-Tzu
AU  - Huang CT
AUID- ORCID: 0000-0002-7337-384X
AD  - Division of Hematology and Oncology, Department of Internal Medicine, Kaohsiung 
      Medical University Hospital, Kaohsiung 807, Taiwan.
FAU - Ou, Tsan-Teng
AU  - Ou TT
AD  - Division of Rheumatology, Department of Internal Medicine, Kaohsiung Medical 
      University Hospital, Kaohsiung 807, Taiwan.
FAU - Hsu, Jui-Sheng
AU  - Hsu JS
AD  - Department of Medical Imaging, Kaohsiung Medical University Hospital, Kaohsiung 
      807, Taiwan.
AD  - Department of Radiology, School of Medicine, College of Medicine, Kaohsiung 
      Medical University, Kaohsiung 807, Taiwan.
FAU - Cheng, Chih-Hung
AU  - Cheng CH
AD  - Division of Pulmonary and Critical Care Medicine, Department of Internal 
      Medicine, Kaohsiung Medical University Hospital, Kaohsiung 807, Taiwan.
FAU - Sheu, Chau-Chyun
AU  - Sheu CC
AUID- ORCID: 0000-0002-7979-3749
AD  - Division of Pulmonary and Critical Care Medicine, Department of Internal 
      Medicine, Kaohsiung Medical University Hospital, Kaohsiung 807, Taiwan.
AD  - Department of Internal Medicine, School of Medicine, College of Medicine, 
      Kaohsiung Medical University, Kaohsiung 807, Taiwan.
LA  - eng
GR  - KMUH108-8R17; KMUH109-9R13/Kaohsiung Medical University Hospital/
PT  - Case Reports
PT  - Review
DEP - 20230210
PL  - Switzerland
TA  - Medicina (Kaunas)
JT  - Medicina (Kaunas, Lithuania)
JID - 9425208
SB  - IM
MH  - Female
MH  - Humans
MH  - Aged
MH  - *Lung Diseases, Interstitial/etiology
MH  - *Connective Tissue Diseases/complications/diagnosis
MH  - *Polymyositis/complications
MH  - Tomography, X-Ray Computed/methods
MH  - Prognosis
PMC - PMC9962511
OTO - NOTNLM
OT  - anti-Jo-1
OT  - glucocorticoid pulse therapy
OT  - interstitial pneumonia with autoimmune features
OT  - polymyositis
COIS- The authors declare no conflict of interest.
EDAT- 2023/02/26 06:00
MHDA- 2023/03/03 06:00
PMCR- 2023/02/10
CRDT- 2023/02/25 03:33
PHST- 2022/12/31 00:00 [received]
PHST- 2023/02/06 00:00 [revised]
PHST- 2023/02/08 00:00 [accepted]
PHST- 2023/02/25 03:33 [entrez]
PHST- 2023/02/26 06:00 [pubmed]
PHST- 2023/03/03 06:00 [medline]
PHST- 2023/02/10 00:00 [pmc-release]
AID - medicina59020330 [pii]
AID - medicina-59-00330 [pii]
AID - 10.3390/medicina59020330 [doi]
PST - epublish
SO  - Medicina (Kaunas). 2023 Feb 10;59(2):330. doi: 10.3390/medicina59020330.