POS1183 DIAGNOSTIC CHALLENGES IN A PREGNANT PATIENT WITH SLE AND SYSTEMIC SCLEROSIS OVERLAP – PREECLAMPSIA, ACTIVE LUPUS NEPHRITIS OR RENAL CRISIS?

Abstract

Background: Pregnancy is an important issue for young women with inflammatory systemic disease and pose a clinical challenge. Objectives: The aim of this case report is to underline the challenges to correctly diagnose pregnancy complications versus disease flare in pregnant women with overlap syndrome. Methods: Clinical information collected from the patient's journal. Results: A 26 years old woman was referred to Karolinska University Hospital because of Raynaud's phenomenon, sclerodactyly, skin rash, palate ulcerations and recurrent finger ulcerations with necrosis, infections and self-amputation of distal phalanges. Interstitial lung disease was confirmed by high resolution CT and a restrictive pattern on lung function tests. Immunological analyses detected autoantibodies against Scl-70, ribosomal-P, SS-A, SS-B, Ku, as well as dsDNA with immunofluorescence technique with varying titers over the years. Complement activation, anemia and lymphopenia were also present. The patient experienced muscle weakness and peripheral muscle MRI and muscle biopsy confirmed myositis. During follow-up she developed pericardial effusion and myocarditis. The patient was diagnosed with overlap syndrome with clinical and serological features of systemic sclerosis, SLE and myositis. The immunosuppressive treatment over the disease course comprised hydroxychloroquine, methotrexate, azathioprine, rituximab and low dose prednisolone. Later, treatment was switched to mycophenolate mofetil because of flares on the previous regimens. For the digital ulcers, she was treated with nifedipine, sildenafil and iloprost infusions. Despite medical advice on pregnancy risks during active disease, the patient stopped medication with mycophenolate at the age of 35 years and became pregnant. Enalapril, spironolactone and sildenafil were discontinued and she was referred to the specialist maternity care. The pregnancy evolved without complications until week 18 when the blood pressure (BP) began to rise. In trying to avoid alpha blockers, because of recent finger ulcerations, nitrates and hydralazine were started without sufficient effect on the BP. Soon thereafter, she also developed marked trombocytopenia and hemolytic anemia. The titers of dsDNA antibodies were higher compared to the status before pregnancy and the creatinine and proBNP started to rise. Urine analysis revealed proteinuria and granular casts. Pre-eclampsia was suspected, though SLE flare with active nephritis, hemolysis and trombocytopenia could not be ruled out. Tacrolimus, intravenous immunoglobulin and methylprednisolone were started, as well as labetalol and diuretics. Plasmapheresis was applied twice. The BP remained uncontrolled and kidney function was deteriorating; therefore hemodialysis was started. Since the foetal growth was impaired and the patient's clinical status was not improving, decision was made to stop the pregnancy. The medical abortion with vaginal birth proceeded without complications and minimal hemorrhage. Since hypertension and hemolysis continued post-abortion despite several antihypertensive drugs and the patient developed lung edema, renal crisis was suspected and the patient was started on enalapril. BP, and later on the renal function, improved slowly. Two weeks postpartum dialysis was no longer needed. Renal biopsy revealed thrombotic microangiopathy without signs of nephritis. Conclusion: We described a patient with overlap syndrome who developed serious pregnancy complications that ended up with pregnancy termination in week 20. This case illustrates the difficulty in differential diagnostics of preeclampsia-induced hypertension with renal and hematological changes versus active SLE and the importance of keeping in mind that renal crisis may complicate the clinical picture in SLE/ scleroderma patients. Also, this case underscores the significance of low disease activity when starting a pregnancy and that every effort should be made for shared decision making.