Systemic Lupus Erythematosus (SLE) in Children

00:01 In this lecture, we're going to discuss lupus, more formally known as systemic lupus erythematosus or SLE.

00:11 Lupus has a unique pathology.

00:15 Basically, this is a multisystem inflammatory autoimmune disease that's caused by immune complex mediated damage.

00:24 It affects multiple organs, in different ways, in different people.

00:28 And the cause is unknown.

00:30 There are genetic factors, environmental factors, and hormonal factors, which mitigate the disease.

00:37 It is more common in girls than boys.

00:41 And the peak pediatric age of diagnosis is 9 to 15 years of age.

00:46 We don't usually see it in the very young children.

00:50 So the pathophysiology of lupus is, as we stated both genetic and environmental.

00:56 In a susceptible individual who has susceptibility genes, they're going to have an event that results in a apoptosis.

01:05 For example, they might have got a sunburn on their skin, a UV radiation.

01:10 A period during which cells are breaking down on a larger scale.

01:16 These patients because of their genetic susceptibility, will have a defective clearance of these apoptotic bodies, resulting in an increase burden of nuclear antigens throughout the body.

01:29 In this susceptible person, the B cells and T cells will mount an immune response against these nuclear antigens.

01:37 This will create anti-nuclear antibody antigen complexes, which will then go through the body going to stimulate both dendritic cells, which then stimulate B cells and create antibodies vastly against nuclear antigens.

01:54 These nuclear antigen with antibody complexes are causing the mainstay of the disease in organs throughout the body.

02:02 So let's go through some of the basic symptoms of SLE or lupus.

02:08 Constitutionally, these patients get frequent fevers, they may have fatigue, and they'll develop weight loss as well.

02:16 Their skin has a few key findings.

02:18 This girl has one of them, a malar rash.

02:22 They may also have a discoid rash, which are little round circles around the skin.

02:28 Discoid rashes are also common in babies with lupus and we'll talk about that entity separately.

02:35 Also, patients may get photo sensitivity, which is a increased susceptibility to burn when exposed to the sun.

02:44 Patients do get lung involvement.

02:46 They can get pleuritis, pleural effusions, pulmonary hemorrhage, or even pulmonary hypertension.

02:52 They have cardiac findings.

02:55 In additionally, they will have pericarditis, myocarditis, and endocarditis.

03:00 Any part of the heart can be evolved, and they can get cardiovascular or vascular problems such as Raynaud's phenomenon, which you can see in this patient here.

03:09 Where there is decreased blood flow to the distal end of a digit.

03:15 In patients with lupus, they can get an endocarditis that is unique called Libman-Sacks endocarditis.

03:22 It looks grossly like bacterial endocarditis, but does not grow any organisms on blood culture.

03:29 This can appear as single or multiple 1 to 3 millimeter warty deposits on those valves and you can see a picture of one here.

03:39 These patients may have involvement of their renal pathways.

03:43 They may develop proteinuria or hematuria depending on if they're having more nephritic or nephrotic syndrome.

03:49 They may develop pyuria from just white blood cells that are inflamed in the kidney, but they will not grow anything on the urine culture.

03:57 They may develop hypertension as a result of their end stage renal disease and they may proceed to renal failure.

04:05 The GI system can get involved as well and in particular, these patients may develop oral ulcers, pancreatitis, hepatitis, intestinal vasculitis or even a protein-losing enteropathy.

04:18 Essentially, any part of the GI tract can be involved.

04:23 They may have muscle disorders, they may develop arthritis or myositis, or a vascular necrosis of a joint.

04:31 And importantly, they may develop brain problems.

04:34 Roughly 10% of patients with lupus will present with a brain problem.

04:40 Examples include stroke, psychosis, seizure, chorea or a tremor, they may develop a transverse myelitis of the spine.

04:52 Additionally, patients can get problems with their blood and any blood line can be affected.

04:57 They may get lymphopenia, anemia, or thrombocytopenia any cell line on the CBC can go down.

05:05 So there are several ways that people can diagnose lupus, and it gets confusing, I think, for a lot of students because we used to have 11 criteria and now there's two different sets of criteria.

05:17 There's the ACR criteria and the SLICC criteria.

05:20 The SLICC criteria are probably more commonly used in children.

05:23 So I want to go through them here.

05:25 To meet criteria you need four clinical criteria.

05:29 And then additionally one immunologic criteria.

05:33 So we can go through them here.

05:35 And the clinical criteria include acute cutaneous lupus, chronic cutaneous lupus, oral or nasal ulcers, Non-scarring alopecia, arthritis, Serositis.

05:47 Remember, the cirrhosis are the lining of the pericardium, the pleura and the peritoneum.

05:52 So any kind of inflation of those services, renal, any renal involvement, any neurologic involvement.

05:59 Keep in mind that about 10% of patients will present with a primary neurologic condition.

06:04 Hemolytic anemia, leukopenia and thrombocytopenia.

06:09 Additionally, patients need to meet an immunologic criteria, and those include a positive ANA, a positive anti DNA, a positive anti Smith antibody, positive antiphospholipid antibody lo complement levels or also a direct Coomb's test, which doesn't count in the presence of hemolytic anemia has to sort of be in addition.

06:32 Keep in mind that the ANA is an interesting test.

06:35 It's commonly a false positive, but virtually never a false negative.

06:40 So a patient doesn't have an ANA it's very unlikely that they have lupus.

06:45 So, this is a lot of stuff.

06:48 These are a lot of symptoms to remember.

06:51 And unfortunately, there's no gene test for lupus.

06:55 it's a clinical diagnosis.

06:57 For a diagnosis, you need 4 or more of the 11 major criteria of lupus.

07:04 Who can remember 11 criteria of lupus? Well, you can with a little pneumonic.

07:09 Here it goes.

07:10 M.D., please offer all RNs a holiday immediately.

07:15 Again, M.D., please offer all RNs a holiday immediately.

07:20 You have to do the S after the RNs.

07:22 Let's look at it this way.

07:24 M.D., please offer all RNs a holiday imediately.

07:29 What's clever about this is the last three are lab criteria.

07:33 And the first eight are body physical exam criteria.

07:38 Let's go through them again so we can remember them.

07:41 M is the malar rash. D is the discoid rash.

07:45 And P is the photosensitivity.

07:48 Those are the skin findings in lupus.

07:51 O is oral ulcers.

07:54 A is arthritis.

07:56 The R is renal involvement, and the N is neurologic involvement.

08:02 The S is tricky. It's Serositis.

08:05 Remember, your serosa are superficial coverings of your organs.

08:10 So the area around the heart is the pericardium that is a serosa.

08:14 So pericarditis is a serositis.

08:17 Peritonitis is a serositis.

08:19 Pleuritis or a pleural effusion is a serositis.

08:24 Next, the labs.

08:26 The ANA test is its own criteria.

08:29 The interesting thing about the ANA test is that virtually every patient with lupus will have a positive ANA.

08:36 This is the one that almost all of them have that is positive.

08:39 So a positive ANA is necessary almost for a diagnosis of lupus, If they're not any positive, they will be very soon.

08:50 The challenge is, is that one in three people walking down the street will have a positive ANA.

08:55 Hack,they're even a little bit infectious.

08:58 And by that I mean, if you are ANA negative and you move into the home of someone who has ANA positive, you're more likely to become ANA positive.

09:07 That doesn't mean you're getting lupus.

09:09 So the ANA positivity is necessary but not sufficient for the diagnosis.

09:16 H is heme labs.

09:18 Again, back to any of those elements of the CBC.

09:21 They may have an abnormal white count, abnormal hemoglobin, or abnormal platelets and all of them are usually low.

09:28 And I is immune labs.

09:31 And I'll walk you through what those are in a bit.