Acute treatment-resistant post-partum necrotising myopathy with anti-Ro52/TRIM21 antibodies treated with rituximab

Abstract

examination of the upper and lower limbs revealed proximal muscle weakness MRC grade 3/5 in both upper and lower limbs. Due to her rapid decline (advancing muscle weakness on a daily basis), a neurological evaluation and a magnetic resonance imaging (MRI) were requested to exclude possible multiple sclerosis. The MRI brain did not show any relevant findings. The MRI spine revealed some congenital abnormalities. An immunological screen showed positive anti-Ro52/TRIM21 antibodies but negative anti-Jo1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, anti-KS, anti-Zo, anti-Ha, anti-Mi-2, anti-MDA5, anti-155/140, anti-140, anti-SAE, anti-SRP, anti-HMGCR (anti 200/100), PM-Scl100, Ku and PM.Scl75 antibodies. Her blood tests showed elevated creatinine phosphokinase (CPK) at 38,040 U/L (normal range 136-145 U/L), lactic dehydrogenase (LDH) at 1,500 U/L (normal range 135-214 U/L), aspartate aminotransferase (AST) at 448 U/L and alanine aminotransferase (ALT) 225 U/L, whilst gamma-glutamyl transpeptidase (γGT), bilirubin and alkaline phosphatase were within normal range. Muscle carnitine levels were normal. The muscle biopsy showed acute non-inflammatory necrotising myopathy. In Fig. 1 below, we can see the initial patient presentation and the histological samples from the muscle biopsy.An electromyography study (EMG) was also conducted. The deltoid, rectus femoris, vastus lateralis, vastus medialis, tibialis anterior and soleus muscles were examined on both sides. The findings were consistent with polymyositis. Whole-body CT scan did not show any abnormal tumour growths. The patient was initially treated with high-dose IV methylprednisolone 1 g for 3 days and showed some minor initial improvement. When she was put on oral steroids, she rapidly deteriorated with more pronounced muscle weakness and dysphagia. Her deterioration was so rapid that she was transferred to the intensive care unit. Additional Sir, a 29-year-old lady presented 2 months post-partum with a 2-week history of hoarse voice, lethargy, progressively worsening proximal myopathy, peri-orbital oedema, rash on her upper chest, hands and face. Initial clinical presentation was that of dermatomyositis and the patient's general condition declined within a week of hospital admission with generalised paralysis and inability to breathe on her own. The patient was not expected to live.The patient had a 5-year history Hashimoto's thyroiditis, for which she was treated with 50 mcg thyroxine once daily. She had no history of smoking or alcohol consumption, nor any known allergies. She had a known thalassaemia trait. She had conceived through in vitro fertilisation treatment and had delivered with a caesarian section, epidural anaesthesia, a healthy baby girl at 38 weeks of gestation. This was a first and only pregnancy. She did not have any other past significant medical history nor any family history of note.Her thyroid peroxidase antibodies were elevated at 62.9 IU/ml, and her thyroglobulin antibodies were within normal range. Her respiratory, gastrointestinal, genitourina...

Successful treatment of seronegative immune‐mediated necrotizing myopathy developing during pregnancy: A case report and literature review

“…There have been only a few reported cases of IMNM during pregnancy. Previously reported cases of IMNM associated with pregnancy are summarized in Table 1 4–10 . Four cases, including the present case, developed and were diagnosed during pregnancy, and three cases developed postpartum.…”

“…Previously reported cases of IMNM associated with pregnancy are summarized in Table 1. [4][5][6][7][8][9][10] Four cases, including the present case, developed and were diagnosed during pregnancy, and three cases developed postpartum. In all three cases of recurrence, treatment was interrupted at the time of pregnancy, suggesting that recurrence due to the interruption of treatment may be more important than exacerbation of symptoms due to pregnancy.…”