A case report of ineffective immunosuppressive therapy for MDA5 related clinically amyopathic dermatomyositis during pregnancy and literature review.

BACKGROUND

Clinically amyopathic dermatomyositis (CADM) is a type of dermatomyositis (DM) with hallmark cutaneous lesions of DM, and no muscle weakness for a prolonged period. Antibodies tests against melanoma differentiation-associated gene 5 (MDA5) was recognized to be highly specific for CADM, and is a high risk of mortality. Immunosuppressive therapy has been reported is effective. We report ineffective immunosuppressive therapy for pregnant woman complicated by CADM with MDA5 positive, which has seldom reported before.

CASE DESCRIPTION: A 43-year-old woman at a gestational age of 21 weeks, gravida 4 para 1, with dyspnea, cough, dizziness and fever, purplish-red rash on her hands, without muscle pain or weakness, was admitted to hospital. Fetal ultrasonography showed normal fetal growth. She denied any family history and had no specific past history. On day 13 of hospitalization (gestational week 22+6), the fetus had died. With the discovery of antibodies against MDA5, purplish-red rash and no muscle involvement since the onset of her condition, she was diagnosed with MDA5 related CADM. Immunosuppressive therapies [methylprednisolone (80 mg/day), methylprednisolone (500 mg/day), gamma globulin (10 g/day), cyclophosphamide (1 g)] and antibacterial drug [ceftriaxone sodium (intravenous drip, 2 g/day), piperacillin tazobactam (intravenous drip, 4.5 g per 8 hour)] may be good at improving her symptom at first, while were ineffective to stop deteriorating of her condition at the end. High-resolution chest computed tomography showed nonspecific interstitial pneumonia (NSIP). The woman died of respiratory failure for life-threatening rapidly progressive interstitial lung disease (RP-ILD) at last.

CONCLUSIONS

MDA5-related CADM is a high fatal disease. Immunosuppressive therapies for MDA5-related CADM may be ineffective. Women with that should conceive extremely prudently. Meanwhile, pregnancy woman with MDA5-related CADM must deserves physician team's attention.