Neonatal Lupus Erythematosus: Understanding Causes, Symptoms, and Management.

Introduction:

Lupus is a rare chronic autoimmune disorder where the immune system functions against its own. The main symptoms could be inflammation, joint pain, skin rashes, and fatigue. The exact cause is unknown, and the diagnosis is tough due to its unpredictability. Different types of lupus include systemic lupus erythematosus, cutaneous lupus, drug-induced lupus, and neonatal lupus. Among these, systemic lupus is common, and the other goes unnoticed. On the other hand, lupus is not contagious. It runs in families and sometimes reacts to environmental Triggers. This article focuses on neonatal lupus erythematosus.

What is Neonatal Lupus?

Neonatal Lupus Erythematosus (NLE) is a rare condition that affects infants born to mothers with certain autoimmune diseases. Neonatal Lupus Erythematosus is used to describe a clinical spectrum of abnormalities seen in newborns' skin, heart, and systemic abnormalities. The disease manifests as a non-scarring, non-atrophic skin lesion. Sometimes, the babies are born without skin lesions, but they develop after a few weeks of birth. There may also be involvement from the pulmonary, central nervous, hematological, hepatobiliary, and cardiac systems.

How Does Disease Affect Newborns?

The main cause of the disease in newborns is the transplacental passage of maternal autoantibodies of the affected mother. Maternal autoantibodies, particularly anti-Ro/SSA, anti-La/ SSB, and less commonly U1 - RNP, cross the placenta and affect the developing fetus. The incidence of NLE is 1-2 % in mothers carrying the autoantibodies, either symptomatic or not. The exact mechanism of how the disease develops in the fetus is not known, but these antibodies can produce lesions in the skin, congenital heart block - affecting the electrical conduction system of the heart in the fetus, liver problems, or low blood count.

What Indicates the Presence of NLE?

The symptoms may come and go, and it varies from person to person. The most common symptom of NLE is skin rash.

1. Skin Rash: NLE is characterized by transient reddish, ring-like skin lesions found in the malar region in the shape of the butterfly-malar rash or around the eyes, giving a Racoon eye pattern. This could be one of the striking features of NLE. Apart from this, the rashes may also be found on the trunk, arms, legs, and scalp. The affected individual exhibits abnormal sensitivity when exposed to light (Photosensitivity). The lesion may develop after 6 weeks of life. In some cases, after 2-3 months of birth, the sign is evident.

2. Congenital Heart Block: This could be a serious complication where the conduction of the heart is impaired in varying degrees. In severe cases, there will be a lag in the conduction system and cause a complete blackout, namely syncope, Breathlessness, and an irregular heartbeat called cardiac arrhythmias. Less often, there will be an increased growth of the cardiac muscle, termed cardiomyopathy, due to the thickening of the muscles in the chambers. This may cause serious complications like myocarditis (Inflammation of the heart muscles), heart failure, and cardiac arrest.

3. Hematopoietic Complications: There will be a reduction in the circulating red blood cells, causing anemia, platelets, and neutrophils (type of white blood cell). This results in fever, fatigue, body pain, and weight loss.

4. Non-Cardiac Abnormalities:

   1. Splenomegaly: Abnormally large spleen.

   2. Hepatomegaly: Enlarged liver.

   3. Hepatitis: Inflammation of the liver.

   4. Jaundice: Yellowish discolouration of the skin and mucous membranes.

   5. Elevated Liver Enzymes: There will be a transient increase in the liver enzymes with no associated symptoms.

Most of the non-cardiac abnormalities resolve on their own when the maternal autoantibodies get eliminated from the infant’s circulation.

How Is the Diagnosis Made?

Diagnosis is the crucial part when it comes to Neonatal Lupus and it purely depends on the manifestation.

During Pregnancy:

• If the pregnant mother is known to have an autoimmune disorder, then the healthcare provider may prescribe an echocardiogram, where the fetal heart rate can be monitored, and echocardiography to picture the heart with the help of sound waves.

• If a Pregnant mother is unaware of the autoimmune disorder, the mother should be tested for the anti-Ro and anti-La antibodies in the blood.

In Children:

The necessary laboratory tests a child must undergo to rule out the autoimmune disorder are:

1. Complete Blood Count (CBC): To check the levels of Red Blood Cells, White Blood Cells, and platelets as it rules out anemia.

2. Antinuclear Antibodies (ANA): This test detects the abnormal proteins that a body’s immune system makes during the defense mechanism. The presence of these antibodies confirms Lupus or other autoimmune conditions.

3. Anti-DNA: Detects thePresence of specific antinuclear antibodies suggestive of lupus.

4. Complement (C3 and C4): Low levels of C3 and C4 indicate Lupus activity and risks for infection.

5. C-Reactive Protein: Increased levels of C - Reactive protein indicates lupus as this protein is produced by the liver.

6. Erythrocyte Sedimentation Rate: This test is done to determine how quickly the lumps form at the bottom of the test tube, which is indicative of inflammation.

How Can the Condition Be Treated?

The treatment should be directed towards the specific symptoms that are evident in the individual.

Cutaneous Symptoms:

• The skin rashes would go on their own during the initial days. The child should be protected from the sunlight by protective clothing or sunscreen lotions in the beginning to prevent photosensitivity.

• Mild topical steroids can be applied to alleviate skin symptoms and rashes. This should be done according to the pediatricians' advice.

Cardiac Symptoms:

The child must undergo an electrocardiogram to assess the severity of the symptoms. If a congenital heart block is found, a pacemaker can be inserted later in childhood, followed by periodic assessment.

Other Symptoms:

To alleviate the pain and other symptoms.

• Antimalarials - prevent the progression of the disease.

• Steroidal Anti-inflammatory - to ease the pain, swelling and inflammation.

• Corticosteroids- to suppress the immune system.

• Biologics - to target the specific sites and ease the symptoms.

• Cytotoxins - Prevent rapid division of the cells.

Apart from the prescribed medicines, some need intravenous administration of Immunoglobulins and antibiotics to treat the complications. Treatment depends purely on how the individual’s condition progresses.

What Are the Tips to Ease the Condition At Home?

Despite taking medicines some home remedies can help to prevent the progression of the disease. They are:

• Avoid exposure to the sunlight unnecessarily. If going under the sun, wear protective clothing.

• Eat a balanced diet. Consuming more vegetables and fruits, whole grains, proteins and healthy fats can promote overall well-being.

• Stay Active by encouraging the child to be involved in physical activities like swimming, cycling, walking etc.

• Take rest when necessary.

Conclusion:

Systemic conditions need a holistic approach to treat and prevent the disease. Periodic monitoring of pregnant women and children is needed to prevent the condition in the developing fetus. As the disease is unpredictable, treatment should be given uniquely.