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Indian Journal of Medical Sciences, Vol. 59, No. 3, March, 2005, pp. 109-112 Case Report A case of autoimmune myopathy in pregnancy Pasrija Shikha, Rana Ritu, Sardana Kabir, Trivedi S.S. Lady Hardinge Medical College and Shrimati Sucheta Kriplani Hospital, New Delhi Code Number: ms05016 ABSTRACT Autoimmune diseases are not found frequently with pregnancy in clinical practice. Polymyositis Dermatomyositis have a prevalence of 2.4-10.7/ 100,000 in general population. This is further low in pregnant women. It is associated with 57% perinatal morbidity and increased maternal and fetal mortality. Literature suggests that pregnancy outcomes are poorer if it manifests early in gestation while development or exacerbation in second or third trimester is associated with a better fetal prognosis. Not many case reports are published where the disease was diagnosed in third trimester. We present a case detected in third trimester, which was initially mistaken as a case of allergic reaction, however timely diagnosis and adequate management resulted in good fetal and maternal outcome.KEYWORDS: Dermatomyositis, Polymyositis, Myopathy, pregnancy INTRODUCTION Polymyositis and dermatomyositis are acquired inflammatory myopathies of autoimmune basis characterized by muscle weakness, elevated levels of serum muscle enzymes and endomysial inflammation. It is a rare disorder, the reported incidence being 1:28000 with a female preponderance.[1] The peak age group affected is in fifth or sixth decade though a smaller peak occurs in teenagers. Only 17% of pregnant females with adult onset disease develop flare-ups.[2] Pregnancy outcome is unfavorably affected in presence of the active disease. This report describes successful management of pregnancy in a woman with active disease. The rarity of the disease may lead to misdiagnosis, which can result in increased morbidity and mortality.CASE REPORT A 27-year-old second gravida with thirty-one weeks of gestation was referred to us in view of a suspected hypersensitivity reaction. She had rashes over the body for past 2 months, and breathlessness, weakness of muscles and difficulty in swallowing for past 10 days. She developed erythematous, itchy rashes over her eyelids and dorsum of hands about 2 months back, which gradually progressed to involve her upper and lower limbs, part of abdomen and back. She gave positive history of photo dermatitis and photosensitivity. She had developed proximal muscle weakness for the past 10 days as suggested by difficulty in combing her hair. She also felt breathless while eating & drinking, and mastication was difficult. She complained of polyarthralgia and palpitations. There was no history of any drug intake or fever. Her first pregnancy was uneventful and she had a caesarian delivery due to fetal distress two years back. There was no history of asthma, allergy or chronic illness. On examination she had a buffalo hump, heliotrope and Gottron rashes. Her power was 4/5 in most proximal muscle groups, compared to distal muscle groups (5/5). Mild pedal edema was present. She was comfortable but slightly dyspnoec. On abdominal examination she was 30 weeks with breech presentation. Her uterus was relaxed. Pfannenstiel scar was present and there was no scar tenderness. Dermatology and medical opinion was taken and a clinical diagnosis of polymyositis and dermatomyositis was made. Serum levels of CPK mB (Creatinine Phosphokinase mB Isoenzyme) were done and were found to be 396 U/L (normal <167 U/L). On further investigations, Erythrocyte sedimentation rate was 50; LDH (Lactate Dehydrogenase) was 124 U/L (normal 180-325 U/L). Serum transaminases were with in normal range. Antinuclear antibodies, Rheumatoid factor antibody, Lupus erythemtosus cell antibody; Anti Ds DNA antibodies were all negative. Electromyography in the right deltoid muscle showed myopathic pattern and muscle biopsy from the deltoid revealed perifascicular infiltration with lymphocytes and necrotic areas. Chest X-ray was normal. She was started on dexamethasone 8mg IM bid, hydroxychloroquine 200 mg bid and antihistamines along with topical steroids. After 7 days patient was symptomatically better but her glucose profile was found deranged. Blood glucose levels were controlled with diet management. Her symptoms were relieved to some extent, though she still had episodes of dyspnoea & palpitations and her rashes persisted. Echocardiography was done at 33 weeks of gestation and it revealed generalized left ventricular hypokinesia with a left ventricular ejection fraction of 45%. Doppler study was done at 36 weeks of gestation, which showed a high resistance index in the left uterine artery (0.6). Elective caesarian was planned at 37 weeks for fetal sake but she went into spontaneous labor a day prior to that. In view of normal ejection fraction, she was allowed for vaginal delivery however scar tenderness was detected during labor and caesarean section was done. Intraoperative and postoperative periods were uneventful. Baby weight was 2.5 kgs, apgar score was 7, 8, 9 and there were no congenital abnormalities. During puerperium her symptoms improved gradually and oral steroids were given (prednisolone 40 mg bid) along with hydroxychloroquine. CPK mB levels returned to normal by 5 weeks postpartum (71U/L). However the skin lesions persisted. She was started on methotrexate 2.5 mg bid while steroids were tapered off and hydroxychloroquine was stopped. She was followed for 8 months postpartum during which her cardioresoiratory and muscular symptoms have completely abated but the skin rashes improved slowly.DISCUSSION Wagner first described Polymyositis dermatomyositis in 1863. Bohan and Peter proposed five diagnostic criteria, which allowed the comparison of various case reports.[3] These include symmetrical limb girdle or anterior neck flexor weakness, muscle biopsy evidence, elevation of skeletal muscle enzymes, electromyographic changes and dermatologic features such as heliotrope rash, Gottron rash etc. Polymyositis dermatomyositis is uncommon in reproductive age group and thus it is rarely seen in pregnant women. In an analysis of published data, disease showed flare-up in 40% pregnant patients with childhood onset disease and 17% pregnant patients with adult onset disease.[2] There are very few cases reported with the onset during pregnancy. Recurrences in subsequent pregnancies are rare.[4],[5]In a review of literature, Silva et al found 27 cases with either active disease or exacerbations during pregnancy and puerperium.[6] Of these, 8 cases developed postpartum activity. The remaining 19 cases were seen during pregnancy. Out of these, outcomes of the seven cases in the first trimester included abortions (3), neonatal death (1), stillbirth (1), preterm (1) and healthy baby (1). Of the 5 cases in second trimester the outcomes were abortion (2), intrauterine growth retardation (1), stillbirth (1), and healthy baby (1). Out of 7 cases with disease activity in third trimester 3 resulted in preterm deliveries, one had intrauterine growth retardation and 3 delivered healthy babies. 22 other patients with inactive disease showed much better fetal prognosis with 16 healthy babies. In another review, active polymyositis-dermatomyositis associated with pregnancy resulted in healthy infants in 30% of cases, premature babies in 30%, and fetal death or abortion in 40%, whereas during inactive or improved polymyositis-dermatomyositis the figures were 57% healthy babies, 21.5% preterm babies , and 21.5% fetal deaths/ abortions.[7] Intrauterine growth retardation and abruption placenta are also reported in literature. Fetal prognosis is thus found to be worse in childhood onset disease, early gestation and active disease. Two cases of maternal mortality have also been reported due to the disease.[8],[9] The women with active disease are known to worsen during the pregnancy unless therapy is instituted. Our initial treatment relied on steroid therapy, which is the first line therapy for treatment of pregnant woman with polymyositis dermatomyositis. It serves dual purpose of controlling the symptoms and promoting the pulmonary maturity of the fetus. Respiratory difficulty and swallowing difficulty in our case was a manifestation of myositis affecting the respiratory and pharyngeal muscles, seen in about 20% affected cases in literature. There was a possibility that ventilatory support may be required or use of 2nd line drugs may become necessary. Second line drugs that may be used are intravenous immunoglobulins (category c), antineoplastic agents and antimetabolites (category d). Most pregnant women respond to corticosteroids and show gradual improvement in 1-3 months. Palliative therapy includes low salt, low carbohydrate and high protein diet to avoid weight gain and hypertension with steroids. For skin rashes immunosuppressive treatment with hydroxychloroquine with or without topical corticosteroids, avoidance of direct sunlight and sunscreens can be used. Non-steroidal anti-inflammatory agents can be added for pain relief, physiotherapy helps to prevent disuse atrophy and joint contractures. Swallowing difficulty may require assistance of speech therapist. Cricopharyngeal myotomy or gastrostomy tube may be required if swallowing difficulty remains a problem. For patients on steroids hydrocortisone should be added at the time of delivery to avoid acute adrenal insufficiency. The correct diagnosis of the disease in pregnancy is essential in order to initiate effective therapy, which is important to prevent fetal mortality and morbidity (46-57%). In order to optimize the pregnancy outcome, pregnancy should be planned in remission. Awareness leading to timely and judicious therapy by treating doctors along with close monitoring of fetal and maternal status by a multidisciplinary team needs to be emphasized. References
Copyright 2005 - Indian Journal of Medical Sciences |
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