Pregnancy outcome in young patient with scleroderma.

Scleroderma is a rare progressive connective tissue disorder that more often affects women in their fourth decade of life. Hence pregnancy is rarely encountered with this disorder. This rare disorder leads to fibrosis that involves skin and various internal organs like lungs, heart, kidneys and gastrointestinal tract. Poor pregnancy outcome is reported in literature. Here a case of scleroderma in a young woman with good pregnancy outcome is reported. Case A 28 year old primigravida presented at 9 weeks of pregnancy with symptoms of pigmentation, thickening and tightening of skin over face and limbs for 5 years with history of painful ulcers over her finger tips for 1 year. USG at 9 weeks was normal with gestational age corresponding to the period of amenorrhoea. On examination she had induration and hyperpigmentation of skin over both upper limbs up to elbow. Digital pitted scars were present. There was no induration over chest, back or lower limbs. Decreased wrinkling of forehead and pinched up nose were present. Oral orifice and oral mucosa were normal (Fig. 1). Dermatology opinion sought with a suspicion of scleroderma was confirmed after skin biopsy. Immunologist’s opinion was taken regarding the management. She was advised to take tab. nifedepine 10 mg twice daily and tab. aspirin 75 mg once daily from 16 weeks of gestation and to continue folic acid. USG at this time did not reveal any fetal anomalies. She was hospitalized and further evaluated at 24 weeks of pregnancy as she complained of difficulty in breathing. X-ray chest P/A taken with abdominal shield did not reveal any evidence of interstitial lung disease (Fig. 2). Haemogram, renal function tests (RFT) and liver function tests (LFT) were normal, 24 h urine protein was less than 150 mg. Anti scleroderma antibodies-70 Ig G were positive. Lupus erythematosus cell (LE), APLA (antiphospholipid antibodies), lupus anticoagulant and anticardilipin antibodies were negative. CPKT-25 IU/l (creatine phosphokinase test). ECHO was normal, USG KUB (kidney urinary bladder) was normal. Fundus did not show any evidence of vasculitis. She could not perform pulmonary function tests (PFT). She was advised to continue tab nifedepine and to take tab deriphyllin retard 300 mg twice daily. Third trimester 2-D USG evaluation was normal. She was hospitalized at 37 weeks of pregnancy and was reevaluated. ECHO, RFT and LFT and were normal. Her haemoglobin was 8.6 gm%. Her height was 141 cm and pelvic assessment showed pelvic outlet contraction. She underwent elective LSCS at 38 weeks under spinal anaesthesia. There were no anaesthetic complications. She was transfused with one unit of packed cells as her post-op Hb was 6 gm%. The neonate was alive, male, weighed Dasari P. (&), Professor Shyjus P., Senior Resident Department of Obstetrics & Gynaecology, JIPMER, Puducherry, India e-mail: [email protected] The Journal of Obstetrics and Gynecology of India (May–June 2013) 63(3):206–208 DOI 10.1007/s13224-011-0068-x