PMID- 32011440
OWN - NLM
STAT- MEDLINE
DCOM- 20200218
LR  - 20220412
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 99
IP  - 3
DP  - 2020 Jan
TI  - Fatal and extensive multiorgan hemorrhages in anti-melanoma 
      differentiation-associated gene 5 antibody-positive dermatomyositis: An autopsy 
      case report.
PG  - e18600
LID - 10.1097/MD.0000000000018600 [doi]
LID - e18600
AB  - INTRODUCTION: Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 
      Ab) is an autoantigen associated with dermatomyositis (DM). Anti-MDA5 Ab-positive 
      DM patients frequently exhibit clinically amyopathic dermatomyositis (CADM), and 
      develop rapidly progressive interstitial lung disease (RPILD). Even with early 
      detection and potent combination immunosuppressive therapy, anti-MDA5 Ab-positive 
      DM patients have a poor prognosis. In the present case report, we present a rare 
      autopsy case of a patient with anti-MDA5 Ab DM with RPILD who exhibited diffuse 
      alveolar damage (DAD) patterning in lung specimens, and extensive hemorrhages in 
      multiple organs. PATIENT CONCERNS: An 82-year-old Japanese man admitted with 
      bacterial pneumonia was subsequently diagnosed with anti-MDA5 Ab-positive DM 
      based on skin manifestations (mechanic's hand, ulcerated palmar papules, and 
      flagellate erythema), myositis, interstitial pneumonia, and elevation of 
      anti-MDA5 Ab titer. DIAGNOSIS: The patient was diagnosed with anti-MDA5 Ab DM, 
      complicated with RPILD. INTERVENTIONS: The patient received potent 
      immunosuppressive therapy consisting of pulse methylpredonisolone at a dose of 
      1000 mg for 3 days, followed by prednisolone at 60 mg/d, a 1000 mg pulse of 
      intravenous cyclophosphamide (IVCY), and oral tacrolimus at 6 mg/d. Intravenous 
      immunoglobulin (IVIG) at a dose of 400 mg/kg/d for 5 days was subsequently 
      administered. OUTCOMES: Despite triple immunosuppressive therapy and IVIG, the 
      patients' respiratory status deteriorated, and the patient died of respiratory 
      failure on the twelfth day after admission. An autopsy revealed pulmonary DAD and 
      multiorgan hemorrhages, including the left iliopsoas muscle, gastric and bowl 
      mucosa, spleen, and left adrenal gland. LESSONS: Multiorgan hemorrhages may be a 
      fatal complication in anti-MDA5 Ab DM patients.
FAU - Watanabe, Tsuyoshi
AU  - Watanabe T
AD  - Department of Rheumatology.
FAU - Takizawa, Naoho
AU  - Takizawa N
AD  - Department of Rheumatology.
FAU - Nagasaka, Toru
AU  - Nagasaka T
AD  - Department of Pathology.
FAU - Nakamura, Yoshihiro
AU  - Nakamura Y
AD  - Department of Rheumatology.
FAU - Ikai, Hiroki
AU  - Ikai H
AD  - Department of Rheumatology.
FAU - Yamamoto, Mari
AU  - Yamamoto M
AD  - Department of Rheumatology.
FAU - Murai, Yukari
AU  - Murai Y
AD  - Department of Pathology.
FAU - Takasugi, Koji
AU  - Takasugi K
AD  - Department of Pathology.
FAU - Yokoyama-Kokuryo, Waka
AU  - Yokoyama-Kokuryo W
AD  - Department of Rheumatology.
FAU - Fujita, Yoshiro
AU  - Fujita Y
AD  - Department of Rheumatology.
AD  - Department of Nephrology, Chubu Rosai Hospital, Minato-ku, Nagoya, Aichi, Japan.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Autoantibodies)
RN  - 0 (Immunosuppressive Agents)
RN  - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1)
RN  - Amyopathic dermatomyositis
SB  - IM
MH  - Aged, 80 and over
MH  - Autoantibodies/immunology
MH  - Autopsy
MH  - Dermatomyositis/*complications/drug therapy/*immunology
MH  - Hemorrhage/*etiology
MH  - Humans
MH  - Immunosuppressive Agents/therapeutic use
MH  - Interferon-Induced Helicase, IFIH1/*immunology
MH  - Lung/physiopathology
MH  - Male
PMC - PMC7220071
COIS- The authors have no conflicts of interest to disclose.
EDAT- 2020/02/06 06:00
MHDA- 2020/02/19 06:00
PMCR- 2020/01/17
CRDT- 2020/02/04 06:00
PHST- 2020/02/04 06:00 [entrez]
PHST- 2020/02/06 06:00 [pubmed]
PHST- 2020/02/19 06:00 [medline]
PHST- 2020/01/17 00:00 [pmc-release]
AID - 00005792-202001170-00011 [pii]
AID - MD-D-19-05234 [pii]
AID - 10.1097/MD.0000000000018600 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2020 Jan;99(3):e18600. doi: 10.1097/MD.0000000000018600.