History

Fact Explanation
Fatigue This is a non-specific complaint which is common to both normal pregnancy and SLE flare. [1,3]
Joint pain Patients with SLE can complain of joint pain. Most of the disease exacerbations cause mild joint pain commonly during the third trimester. [1,3]
Skin rash Appearance of lupus skin rash can be seen during SLE flares. [1,3]
Fever Fever is another presentation of SLE. [3]
History of Raynaud phenomenon Raynaud phenomenon refers to the vasospasm which occurs in the extremities. Patients complain of pain, pallor and cyanosis when they touch cold water. [4]
Symptoms of venous thrombo-embolism Venous thrombo-embolism is a common complication of SLE. It is due to hypercoagulability. Deep vein thrombosis can cause acute severe limb pain. Cough, hemoptysis and acute chest pain are symptoms of pulmonary embolism. Sudden paralysis and neurological impairment can be associated with thrombotic stroke. [1]
Symptoms of preeclampsia Pre-eclampsia can occur due to recurrent thrombosis of the placental bed. Pregnant ladies present with record of high blood pressure, visual disturbances, proteinuria, generalized body swelling and seizures. [1]
Primary subfertility Females with SLE can have primary ovarian failure due to long term use of cyclophosphamide. [1]
History of recurrent miscarriages Patients with SLE are at risk of recurrent fetal losses. [1,2,5]
Symptoms of neonatal lupus Newborns born to mothers with anti-Ro and anti-La antibodies. Cutaneous lesions resemble annular erythematous or polycystic plaques. These lesions may or may not have fine scales and appear predominately on the scalp, neck, or face. Involvement of the periorbital skin causes “raccoon eye” appearance. However skin lesions are not uncommon over the trunk and extremities. Telangiectasia and photosensitivity is also common in neonatal lupus. [6]
References
  1. IOZZA I, CIANCI S, DI NATALE A, GAROFALO G, GIACOBBE AM, GIORGIO E, DE ORONZO MA, POLITI S. Update on systemic lupus erythematosus pregnancy J Prenat Med [online] 2010, 4(4):67-73 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279186
  2. GEORGIOU P. E., POLITI E. N., KATSIMBRI P., SAKKA V., DROSOS A. A.. Outcome of lupus pregnancy: a controlled study. Rheumatology [online] 2000 September, 39(9):1014-1019 [viewed 25 August 2014] Available from: doi:10.1093/rheumatology/39.9.1014
  3. ERGIN RN. Pregnancy-associated systemic lupus erythematosus. Proc (Bayl Univ Med Cent) [online] 2014 Jul, 27(3):221-2 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24982567
  4. MENéNDEZ A, GóMEZ J, CAMINAL-MONTERO L, DíAZ-LóPEZ JB, CABEZAS-RODRíGUEZ I, MOZO L. Common and specific associations of anti-SSA/Ro60 and anti-Ro52/TRIM21 antibodies in systemic lupus erythematosus. ScientificWorldJournal [online] 2013:832789 [viewed 25 August 2014] Available from: doi:10.1155/2013/832789
  5. HENDAWY SF, ABDEL-MOHSEN D, EBRAHIM SE, EWAIS H, MOUSSA SH, KHATTAB DA, MOHAMED NA, SAMAHA HE. Pregnancy related complications in patients with systemic lupus erythematosus, an egyptian experience. Clin Med Insights Reprod Health [online] 2011 May 12:17-24 [viewed 25 August 2014] Available from: doi:10.4137/CMRH.S6862
  6. LUN HON KAM, LEUNG ALEXANDER K. C.. Neonatal Lupus Erythematosus. Autoimmune Diseases [online] 2012 December, 2012:1-6 [viewed 25 August 2014] Available from: doi:10.1155/2012/301274

Examination

Fact Explanation
Blood pressure Pregnancy induced hypertension is a commonly encountered complication of SLE during pregnancy. [1]
Generalized edema Females can develop lower limb edema towards the latter part of the pregnancy, however generalized edema should be evaluated carefully as it can be associated with pregnancy induced hypertension and with lupus nephritis. [1]
Skin rash Females can have skin rash during disease exacerbations. Disc shaped, round and erythematous lesions are characteristic of discoid lupus and later they may cause scarring. Photosensitive rashes can also be seen in some patients. Neonatal lupus can result in skin rash and photosensitivity in the newborn as well. [1,4]
Signs of pulmonary hypertension Diagnosed patients with SLE and pulmonary hypertension should be advised not to be pregnant. Palpable second heart sound, loud pulmonary component of the second heart sound are indicative of pulmonary hypertension. [1]
Focal neurological signs Patients with thrombotic stroke can have hemiplegia, hemiparesis or hemianesthesia.
Acute synovitis Joint swelling and tenderness can be elicited in patients with acute synovitis. This is one of the factors indicating active disease. [2]
Sings of preeclampsia Preeclampsia is a common complication associated with SLE. Apart from generalized edema, patients can have increased reflexes and elevated blood pressure. [3]
Neonatal bradycardia Neonatal bradycardia can be detected due to congenital heart block. Third degree heart block is usually irreversible. [4]
References
  1. IOZZA I, CIANCI S, DI NATALE A, GAROFALO G, GIACOBBE AM, GIORGIO E, DE ORONZO MA, POLITI S. Update on systemic lupus erythematosus pregnancy J Prenat Med [online] 2010, 4(4):67-73 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279186
  2. GEORGIOU P. E., POLITI E. N., KATSIMBRI P., SAKKA V., DROSOS A. A.. Outcome of lupus pregnancy: a controlled study. Rheumatology [online] 2000 September, 39(9):1014-1019 [viewed 25 August 2014] Available from: doi:10.1093/rheumatology/39.9.1014
  3. HENDAWY SF, ABDEL-MOHSEN D, EBRAHIM SE, EWAIS H, MOUSSA SH, KHATTAB DA, MOHAMED NA, SAMAHA HE. Pregnancy related complications in patients with systemic lupus erythematosus, an egyptian experience. Clin Med Insights Reprod Health [online] 2011 May 12:17-24 [viewed 25 August 2014] Available from: doi:10.4137/CMRH.S6862
  4. LUN HON KAM, LEUNG ALEXANDER K. C.. Neonatal Lupus Erythematosus. Autoimmune Diseases [online] 2012 December, 2012:1-6 [viewed 25 August 2014] Available from: doi:10.1155/2012/301274

Differential Diagnoses

Fact Explanation
Antiphospholipid syndrome Patients with antiphospholipid syndrome can present with a history of recurrent miscarriages and with pregnancy induced hypertension. Often these two conditions can coexist. [1]
Congenital thrombophilia Congenital causes of thrombophilia include antithrombin III deficiency, protein C deficiency and protein S deficiency. Patients present with recurrent deep vein thrombosis and thrombotic complications. [2]
Paroxysmal nocturnal hemoglobinuria (PNH) Patients with PNH are susceptible for venous thrombosis in addition to hemolytic anemia and bone marrow failure. [3]
Thrombotic thrombocytopenic purpura [TTP] TTP should also be considered as a cause of thrombocytopenia seen in SLE. [4]
Seborrheic dermatitis Seborrheic dermatitis should be considered as a possible differential diagnosis in neonates presenting with skin rash. This is a chronic and relapsing dermatitis, which commonly involves the scalp and face. [5]
Congenital syphilis Generalized body rash, fever, hepatosplenomegaly and jaundice can be detected in congenital syphilis. [5,6]
References
  1. DI PRIMA FA, VALENTI O, HYSENI E, GIORGIO E, FARACI M, RENDA E, DE DOMENICO R, MONTE S. Antiphospholipid Syndrome during pregnancy: the state of the art J Prenat Med [online] 2011, 5(2):41-53 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279165
  2. CHAN M. Y., ANDREOTTI F., BECKER R. C.. Hypercoagulable States in Cardiovascular Disease. Circulation [online] December, 118(22):2286-2297 [viewed 25 August 2014] Available from: doi:10.1161/​CIRCULATIONAHA.108.778837
  3. BRODSKY R. A.. How I treat paroxysmal nocturnal hemoglobinuria. Blood [online] December, 113(26):6522-6527 [viewed 25 August 2014] Available from: doi:10.1182/blood-2009-03-195966
  4. GEORGE J. N.. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood [online] December, 116(20):4060-4069 [viewed 25 August 2014] Available from: doi:10.1182/blood-2010-07-271445
  5. LUN HON KAM, LEUNG ALEXANDER K. C.. Neonatal Lupus Erythematosus. Autoimmune Diseases [online] 2012 December, 2012:1-6 [viewed 25 August 2014] Available from: doi:10.1155/2012/301274
  6. GUPTA R, VORA RV. Congenital syphilis, still a reality Indian J Sex Transm Dis [online] 2013, 34(1):50-52 [viewed 25 August 2014] Available from: doi:10.4103/0253-7184.112941

Investigations - for Diagnosis

Fact Explanation
Anti-dsDNA antibodies Elevated levels of anti-dsDNA antibodies can be detected in patients with SLE. [1,2,3]
Complement levels Complement levels are usually elevated in patients with SLE, but normal pregnancy can also cause elevated complement levels limiting its usefulness in diagnosis and follow up of the patients with SLE. Variation of C3 and C4 levels is a better indicator than the total complement levels. [1]
Antinuclear antibody Presence of antinuclear antibodies favors the diagnosis of SLE. [4]
References
  1. IOZZA I, CIANCI S, DI NATALE A, GAROFALO G, GIACOBBE AM, GIORGIO E, DE ORONZO MA, POLITI S. Update on systemic lupus erythematosus pregnancy J Prenat Med [online] 2010, 4(4):67-73 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279186
  2. WICHAINUN R, KASITANON N, WANGKAEW S, HONGSONGKIAT S, SUKITAWUT W, LOUTHRENOO W. Sensitivity and specificity of ANA and anti-dsDNA in the diagnosis of systemic lupus erythematosus: a comparison using control sera obtained from healthy individuals and patients with multiple medical problems. Asian Pac J Allergy Immunol [online] 2013 Dec, 31(4):292-8 [viewed 25 August 2014] Available from: doi:10.12932/AP0272.31.4.2013
  3. KEISERMAN B, RONCHETTI MR, MONTICIELO OA, KEISERMAN MW, STAUB HL. Concomitance of IgM and IgG anti-dsDNA Antibodies Does Not Appear to Associate to Active Lupus Nephritis. Open Rheumatol J [online] 2013:101-4 [viewed 25 August 2014] Available from: doi:10.2174/1874312901307010101
  4. AL-SHAMAHY HA, DHAIFALLAH NH, AL-EZZY YM. Clinical and laboratory manifestations of yemeni patients with systemic lupus erythematosus. Sultan Qaboos Univ Med J [online] 2014 Feb, 14(1):e80-7 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24516759

Investigations - Fitness for Management

Fact Explanation
Fetal ultrasound scan Fetal blood supply can be compromised due to recurrent thrombotic events in SLE. So it is important to assess the placental perfusion with Doppler flow. This should be done in 20th and 24th week of gestation. Diminished placental perfusion can be seen in pregnancy induced hypertension. Presence of persistently high resistance to placental flow and presence of an early diastolic notch are radiological signs of increased risk of pre-eclampsia. Assessment of fetal growth is also important as it allows early diagnosis of intra-uterine growth retardation. In the presence of anti-Ro or anti-La antibodies monitoring of the fetal cardiac activity is necessary as they are at high risk of congenital heart block. [1]
Renal function test Assessment of the renal function is indicated in patients with SLE before planning pregnancy as well as during the period of pregnancy. Elevated serum creatinine and altered electrolyte balance will indicate deranged renal function. Glomerular filtration rate is also helpful in assessing the renal function. [1,2]
References
  1. IOZZA I, CIANCI S, DI NATALE A, GAROFALO G, GIACOBBE AM, GIORGIO E, DE ORONZO MA, POLITI S. Update on systemic lupus erythematosus pregnancy J Prenat Med [online] 2010, 4(4):67-73 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279186
  2. MARTíNEZ-MARTíNEZ MU, MANDEVILLE P, LLAMAZARES-AZUARA L, ABUD-MENDOZA C. CKD-EPI is the most reliable equation to estimate renal function in patients with systemic lupus erythematosus. Nefrologia [online] 2013 Jan 18, 33(1):99-106 [viewed 25 August 2014] Available from: doi:10.3265/Nefrologia.pre2012.Jun.11101

Investigations - Followup

Fact Explanation
Full blood count Anemia and thrombocytopenia are common hematological complications associated with SLE. [1,2]
Urine for protein Proteinuria can be associated with SLE nephritis and also with pregnancy induced hypertension. So urine should be regularly checked for the presence of proteinuria. [1]
Anti-dsDNA antibodies Elevated levels of antidsDNA antibodies reflects the increased risk of disease exacerbations and fetal prematurity. However anti-dsDNA antibodies are usually high during the pregnancy, lowering its sensitivity in the assessment of disease activity. [1]
Anti-phospholipid antibodies (aPL) Presence of aPL antibodies reflects increased risk of thrombosis, pre-eclampsia and fetal loss. [1]
Anti-Ro and anti-La antibodies Presence of any of these antibodies indicates high risk of congenital heart block of the fetus. Neonates with congenital lupus also shows elevated levels of these antibodies. [1,3]
References
  1. IOZZA I, CIANCI S, DI NATALE A, GAROFALO G, GIACOBBE AM, GIORGIO E, DE ORONZO MA, POLITI S. Update on systemic lupus erythematosus pregnancy J Prenat Med [online] 2010, 4(4):67-73 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279186
  2. AL-SHAMAHY HA, DHAIFALLAH NH, AL-EZZY YM. Clinical and laboratory manifestations of yemeni patients with systemic lupus erythematosus. Sultan Qaboos Univ Med J [online] 2014 Feb, 14(1):e80-7 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24516759
  3. GUPTA R, VORA RV. Congenital syphilis, still a reality Indian J Sex Transm Dis [online] 2013, 34(1):50-52 [viewed 25 August 2014] Available from: doi:10.4103/0253-7184.112941

Investigations - Screening/Staging

Fact Explanation
Echocardiogram Cardiac activity of the infant should be assessed with an echocardiogram between 18 and 30 weeks of age, due to the risk of congenital heart block. [1]
ECG Neonatal lupus with congenital heart block can be diagnosed with ECG of the newborn. Irregular heart beat and prolonged QT interval are signs indicative of heart block. [2]
References
  1. IOZZA I, CIANCI S, DI NATALE A, GAROFALO G, GIACOBBE AM, GIORGIO E, DE ORONZO MA, POLITI S. Update on systemic lupus erythematosus pregnancy J Prenat Med [online] 2010, 4(4):67-73 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279186
  2. LUN HON KAM, LEUNG ALEXANDER K. C.. Neonatal Lupus Erythematosus. Autoimmune Diseases [online] 2012 December, 2012:1-6 [viewed 25 August 2014] Available from: doi:10.1155/2012/301274

Management - General Measures

Fact Explanation
Health education All diagnosed females with SLE who are planning pregnancy should be well educated about the disease and disease related fetal and maternal complications. Throughout the period of pregnancy they should be followed up closely and preferably in a tertiary care unit. It is better to plan the pregnancy during a period of disease quiescence for at least 6 months, as this improves fetal and maternal outcome. Until then they should be on reversible contraceptive method preferably barrier methods, progestin-only methods or intrauterine devices (IUD). Females with pulmonary hypertension and renal failure should be on permanent contraceptive method as pregnancy is contraindicated. [1,2]
Analgesics Paracetamol and codeine-based analgesia are preferred for relief of joint pain. [1]
Management of hypertension Since most of the commonly used antihypertensives are contraindicated during pregnancy, methyldopa, nifedipine and labetalol should be used in the treatment. [1]
References
  1. IOZZA I, CIANCI S, DI NATALE A, GAROFALO G, GIACOBBE AM, GIORGIO E, DE ORONZO MA, POLITI S. Update on systemic lupus erythematosus pregnancy J Prenat Med [online] 2010, 4(4):67-73 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279186
  2. GEORGIOU P. E., POLITI E. N., KATSIMBRI P., SAKKA V., DROSOS A. A.. Outcome of lupus pregnancy: a controlled study. Rheumatology [online] 2000 September, 39(9):1014-1019 [viewed 25 August 2014] Available from: doi:10.1093/rheumatology/39.9.1014

Management - Specific Treatments

Fact Explanation
Low dose aspirin Low dose aspirin is effective in preventing vascular thrombosis and reducing the incidence of fetal loss. Clopidogrel is another alternative which is considered safe to use during pregnancy. Thromboprophylaxis should be continued for about 4 to 6 weeks after pregnancy in patients with comorbid antiphospholipid syndrome or positive aPL antibodies. [1]
Anticoagulation Anticoagulation with heparin is indicated in patients with SLE. Warfarin is better avoided during early pregnancy, during the period of organogenesis. [1]
Hydroxychloroquine [1] This is an antimalarial drug which is also used in the treatment of SLE. It also acts as a steroid-sparing drug, and an immune modulator. Hydroxychloroquine protects against flares. [1]
Low dose steroids Steroids (prednisolone, dexamethasone, betamethasone) are safe to use during pregnancy. Increased risk of infections, maternal hypertension, gestational diabetes mellitus, osteoporosis, and avascular necrosis of the hip are possible maternal complications that can occur with the use of steroids. Premature rupture of membranes leading to increased risk of infections and premature labor and intra-uterine growth retardation are possible fetal complications of steroid use. Corticosteroid is the drug of choice for the treatment of lupus nephritis. Serositis usually responds to low dose steroids. Severe disease (renal or neuropsychiatric involvement, cutaneous vasculitis) is treated with high dose steroids or with intravenous pulses of (250 or 500 mg) methylprednisolone. [1]
Azathioprine [1] Azathioprin is an immune modulator which is safe to use during pregnancy.
Cyclosporin A [1] Azathioprin is immunosuppressant drug which reduces the immune reaction.
Treatment of skin and joint involvement Nonsteroidal anti-inflammatory drugs (NSAIDs), low-dose prednisolone or hydroxychloroquine is used for the treatment of skin and joint disease. NSAIDs should not be used after 34 weeks of gestation to avoid the possible risk of premature closure of the ductus arteriosus. [1]
Management of neonatal lupus Neonates with congenital heart block and symptomatic bradycardia may need an implantation of a permanent pacemaker. If the neonate has photosensitivity exposure to sun light should be avoided if possible and sunscreen cream should be applied before being exposed to sun light. Telangiectasia, if not settled spontaneously can be treated with laser therapy. Systemic corticosteroids, intravenous immunoglobulin, and/or immunosuppressive agents may be necessary in infants with severe hepatic and hematological involvement. [2]
References
  1. IOZZA I, CIANCI S, DI NATALE A, GAROFALO G, GIACOBBE AM, GIORGIO E, DE ORONZO MA, POLITI S. Update on systemic lupus erythematosus pregnancy J Prenat Med [online] 2010, 4(4):67-73 [viewed 25 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279186
  2. GUPTA R, VORA RV. Congenital syphilis, still a reality Indian J Sex Transm Dis [online] 2013, 34(1):50-52 [viewed 25 August 2014] Available from: doi:10.4103/0253-7184.112941