How long does it take for a person to accept death?

"How long does it take to determine that a sober young woman is bound to die? How long does it take for a person to accept death?" This sentence is a case shared by the "emergency werewolf" that the author saw a few days ago
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This patient suffers from anti-MDA5-positive dermatomyositis with rapidly progressive pulmonary interstitial disease

Let's get to know this disease with the author.
I hope that everyone will never encounter such a patient
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Melanoma differentiation associated gene 5 ( MDA5 ), also known as IFIH 1 (interferon induced with helicase C domain 1), belongs to the RIG-1 (retinoic acid.
inducible gene-I)-like receptor family It is an important member of, plays an important role in the mammalian innate immune response to RNA virus infection , mainly recognizing picornavirus and long dsRNA .

Melanoma differentiation associated gene 5 ( MDA5 ), also known as IFIH 1 (interferon induced with helicase C domain 1), belongs to the RIG-1 (retinoic acid.

inducible gene-I)-like receptor family It is an important member of, plays an important role in the innate immune response of mammals to RNA virus infection , mainly recognizing picornavirus and long dsRNA .

Dermatomyositis (dermatomyositis, DM) is an autoimmune connective tissue disease that mainly affects the skin and muscles

Interstitial lung disease (ILD) isone of the main causes of complications and deathin dermatomyositis (DM) and polymyositis (PM )

ILD patients with positive anti-MDA-5 antibodies usually progress rapidly, and conventional treatment with high-dose glucocorticoids, cyclosporine , tacrolimus, and cyclophosphamide is ineffective

Studies have reported that even with conventional treatment, the mortality rate at 6 months still reaches 60%

Rituximab Rituximab-rituximab 

Tofacitinib—Ithas been reported that the use of Janus kinase (Janus kinase, JAK) inhibitor tofacitinib is effective inpatients with MDA-5 positive ILD after failure of conventional immunosuppressive therapy
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An open study continuously included ILD patients with positive anti-MDA-5 antibodies and were treated with the JAK inhibitor tofacitinib (5 mg, twice a day), and compared with historical controls

Method for the cloth Tropsch Tropsch process for cloth-Tropsch process for the cloth cyclosporine mycophenolate mofetil cyclophosphamide, azathioprine 

Basiliximab-Basiliximab is a monoclonal antibody that antagonizes the interleukin-2 (IL-2) receptor complex alpha chain (CD25), and therefore can interfere with the replication of T lymphocytes and B lymph.

Activation of cells

Basiliximab basiliximab  -  basiliximab prednisone cyclosporine

Other therapies  -It  has been reported that the addition of other immunosuppressive therapies is effective
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An ILD patient with positive anti-MDA-5 antibody received high-dose prednisone , cyclophosphamide, and cyclosporine treatment, and his condition continued to progress rapidly, and thedeterioration did not stopuntil plasma exchange began

Other therapies Other therapies  —  prednisone, cyclophosphamide, cyclosporine, plasma exchange, plasma exchange

Patients with suitable conditions for lung transplantation with severe or progressive lung disease (eg, under 70 years of age and no coexisting diseases) can be referred to a transplant center
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In a small case series study, 5 patients received lung transplantation due to inflammatory myopathy-related ILD; 3 were double-lung transplantation, and 2 were single-lung transplantation

Lung transplant

Diabetes patients with anti-MDA5+ antibody, anti-Ro-52 antibody positive and ILD may delay diagnosis and treatment due to the lack of attention by the patient or atypical symptoms when the rash appears; on the other hand, the rapid progress of the disease and the existence of treatment Resistance, inaccurate effects of current treatment methods and other reasons lead to poor prognosis and high patient mortality
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Therefore, in clinical work, we should do a good job in popular science and pay attention to the screening and monitoring of anti-MDA5 antibodies, pay attention to the status of serum ferritin, EBV and HCMV-IgM antibodies, so as to achieve early diagnosis, early intervention, and strict prevention of various types of infections.
Active immunosuppressive agents and biological agents are combined to delay the progression of the disease as much as possible and improve the prognosis of patients
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