By Dr. Jacqueline Shibata
A 35 y/o with Lupus presents to the ED with 1 day of chest pain…. The worst because, as we all know, systemic lupus erythematous (SLE) is an autoimmune rheumatologic disease that can be deadly in young women.
Ask yourself: Is this just a bad lupus flare with pleuritis causing her chest pain or does this patient have some other complication like an MI from years of steroid use and renal failure even though she’s only 35. Is her hemoptysis from a giant PE, should you anticoagulate if you can’t get that CTA since she has lupus nephritis. Should you just treat empirically or is she having a flare with vasculitis causing her to hemorrhage in to her lungs? Still want to anticoagulate?
Scary complications from this disease include (but aren’t limited to):
–Â Â Â Â Â Â Â Â Â Infections from immunosuppression (fungal, viral, septic arthritis, etc, etc)
–Â Â Â Â Â Â Â Â Â Pulmonary hemorrhage
–Â Â Â Â Â Â Â Â Â Massive PE
–Â Â Â Â Â Â Â Â Â Pericarditis
–Â Â Â Â Â Â Â Â Â AMI
–Â Â Â Â Â Â Â Â Â Side effects from medications
–Â Â Â Â Â Â Â Â Â Libman-Sacks Endocarditis
–Â Â Â Â Â Â Â Â Â Scary microangiopathic badness
–Â Â Â Â Â Â Â Â Â Adrenal insufficiency after a long course of steroids
The list goes on and on… but this is not a review of lupus, although you should go review it (because its so fascinating and kills young people very quickly). These are just some thoughts from a person who occasionally works upstairs.
Emergency physicians are masters of resuscitation, but we don’t always know the exact diagnosis nor do we need to. However, to effectively treat these SLE patients who are set up for a variety of badness, someone needs to be able to make the appropriate diagnosis once the patient is stable.
Here’s a short list of lupus labs that are going to be helpful in making your diagnosis of an active SLE flare:
CBC
– Hemolytic anemia, thrombocytopenia and lymphopenia (<1500)
ESR/CRP
Ua with sediment
Urine Protein and Creatinine (“spot” or “random”)
– Works as surrogate for 24 hour protein. You’re sending urine anyway! This becomes extremely useful for not only diagnosing a flare, but you may save some kidneys and lives)
BUN/Cr
Anti-ds DNA
– These levels will actually increase with flare
Complement levels (C3, C4, C50)
– Low in flare
Some of these labs wont come back in the ED, but by sending them, you may prevent a deadly nosocomial infection in a 30 year old girl by shaving days off her hospital stay (or 2 weeks if the original diagnosis wasn’t made because she was started on IV steroids before getting the diagnostic labs that she needs and now no one knows if it was a flare or she has some occult infection, the work up drags out forever and then she does get an infection………. You get it).
So you have a very young woman with chest pain. Before you say pleuritis and admit…
What about acute MI?
Acute coronary syndrome is a leading cause of morbidity and mortality in women with SLE (up to 40%). Korkmaz et al. notes that young women with SLE who present with chest pain are less likely to have a cardiac work-up and are often misdiagnosed with pleuritis or pericarditis (2).
They have many risk factors for ACS including prolonged steroid use, renal failure, chronic inflammation, underlying vascular injury and predisposition to thrombosis.
One study compared over 400 woman with SLE and MI and women without SLE
from the Framingham Offspring Study and found that MI was more than 50x more common in women with SLE in the 35-44 year old age group!
Treatment is the same as in patients with ACS who do not have SLE (3).
Key point for emergency physicians:
Young women with SLE who have chest pain should be taken seriously even if no active lupus flare. Send a Trop and do an EKG.
For some EKG fun, check out: STEMI vs. Pericarditis: http://lifeinthefastlane.com/ecg-library/basics/pericarditis/
What about PE?
SLE is highly correlated to anti-phospholipid syndrome. Has she ever had a miscarriage?
A 20 year cohort study showed that up to 50% of a patients with SLE have antiphospholipid antibodies. Of those patients, 50-70% will develop DVTs/PEs or have Catastrophic Antiphospholipid Syndrome (A vaso-occlusive crisis that leads to multiorgan failure with a mortality rate of 50% (4)).
These folks should be on life long anticoagulation. If they have ever had a VTE in the past, look for it again. There are 3 specific antibodies to look for, but they don’t need to be sent from the ED especially since you’re so busy sending her spot Prot/Cr which brings me to my last point…
Renal Disease
It is extremely common in SLE and leads to early death, many complications and the medication side effects can be devastating. BUT we can quantify how bad the renal disease is in the ED!
Urine Prot/Cr > 0.5 means the patient is losing greater than 500mg protein/day. She needs a biopsy and some serious medications… now.
Lupus nephritis (LN) can quickly progress to ESRD or can stay clinically silent while the kidney are slowly and irreversibly being damaged. A cohort of 700 SLE patients found that life expectancy in those with renal disease was decreased by 15 years and 24 years if patient had renal damage (5).
Diagnosis and treatment is determined by renal biopsy. Another study showed that a delay from diagnosis of nephritis to time of biopsy independently increased risk for ESRD (6). The medications to treat LN can have disastrous side effects and therefore should be used only after appropriate diagnosis.
Key point for emergency physicians:
Lupus Nephritis is bad. If they have greater than 500mg proteinuria, they should get a biopsy so that they are properly treated. In the meantime, they will likely need to be started on pulsed steroids (good thing you already sent all the lupus labs so that the correct diagnosis will be made).
My main hopes and dreams for the next time you have a lupus patient:
1. Â Â Â You are quickly able to decide if you think this is a flare or a different, but equally serious complication of lupus.
2.       Keep your differentials broad.
3. Â Â Â Â Â Â Send those simple labs so you can dispo the patient correctly.
4.      Even when the labs don’t help disposition (although they always will because you now have a better taste for the flavor of badness she has) these labs are still extremely helpful for the remainder of your patient’s hospital course.
Extra academic fun: Review SOAP BRAIN MD, its really only a couple ROS questions + those labs listed above and you’ll have a quick SLE flare diagnosis or rule out in no time! http://emedicine.medscape.com/article/332244-workup.
References:
1. Manzi, S. et al. Age specific Incidence Rates of MI and Angina in Women with SLE: Comparison with the Framingham Study. Am J. Epidemiology (1997) 145 (5) 408-415. PMID: 9048514
2. Korkmaz C, Cansu DU, Kasifoglu T: Myocardial infarction in young patients (≤35 years of age) with systemic lupus erythematosus: a case report and clinical analysis of the literature. Lupus 16: 289, 2007. PMID 17439937 Â
3. Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Eng J Med. 2002; 346:752-63.
4. Morabito GC, Tartaglino B. Chapter 279. Emergencies in Systemic Rheumatic Diseases. Tintinalli’s Emergency Medicine: A Comprehensive Study Guide. 7e. New York, NY: McGraw Hill; 2011
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2 Comments
Ian deSouza · August 26, 2014 at 4:58 pm
We are doing tons of CTPAs (for PE) on these patients. Should we not be doing so, even with a normal crn? Does a urine protein/creatinine > 0.5 preclude a CTPA?
Can those other blood tests you would like us to send reliably rule in/out an exacerbation? Using what criteria?
Nordstrom · August 27, 2014 at 11:36 pm
I know that there is no single, or combination, of blood tests that you can send to completely “rule out” a lupus flair. Mostly because there are so many different manifestations of the disease both clinically and by pathophysiology. The ds DNA level tends to be elevated in active lupus nephritis and vasculitis, and the titer tends to correlate with disease severity. But even for active lupus nephritis alone an elevated ds DNA titer has a sensitivity of 75% for. Cant rule out.
A positive ds DNA titer does have a good specificity. (ie its likely lupus) And if you have 4 of the 11 (SOAP BRAIN MD) criteria the specificity is 95%.
The same goes for the C3,C4 and ESR/CRP. They are good for tracking disease severity and flairs but are not hard “rule out” tests. I would say send these tests on patients that are going to be admitted for lupus, or if they have a rheumatologist and you can arrange follow up in a few days as these are regularly used to track disease severity and make management decisions.
Regarding the CTA’s in patients with lupus nephritis its a good question. I couldn’t find any definite answer. I think anyone with signs of active or symptomatic nephritis (hematuria, peripheral edema, nephrotic syndrome) with a NORMAL creatinine I would opt to treat empirically for PE and get the VQ scan. If a patient has asymptomatic nephritis (elevated urine protein > 500mg/day) and you need to rule out PE, I don’t think this is a contraindication to IV contrast.
Thanks for the great review Dr Shibata!