Diagnose and treat lupus in primary care with tips from rheumatologist, Beth Jonas MD, FACR (UNC). We discuss the history and exam findings in lupus, initial lab workup, the dreaded ANA, who needs expanded lab testing, lifestyle factors in lupus, vaccinations, the basics of treatment…and Jethro Tull?!
Written and Produced: Kate Grant MBChB DipGUMed; Matthew Watto MD, FACP
Infographic: Matthew Watto MD, FACP
Cover Art: Kate Grant MBChB DipGUMed
Hosts: Matthew Watto MD, FACP; Paul Williams MD, FACP
Editor: Matthew Watto MD, FACP
Guest: Beth Jonas MD, FACR
Painless mouth ulcers should raise the suspicion for lupus. Common aphthous ulcers are painful.
ANA titers less than 1:80 can probably be ignored. ANA titers above 1:80 must be interpreted in clinical context e.g. a positive titer in the presence of hematuria, rash and cytopenias is very suggestive of lupus. –expert opinion
A positive ANA should be followed by a thorough search of the medication list for possible drug induced lupus (hydralazine, procainamide, INH, minocycline).
Initial workup for lupus: In addition to a careful history and physical exam (i.e. skin, joints, mouth), Dr. Jonas sends a CBC, TSH and urinalysis in patients with vague symptoms that might be rheumatologic. –expert opinion
High suspicion for lupus: Send ANA, ENa panel (varies by hospital but might contain anti-Smith, Ro/La, anti-Jo1, SCL-70, RNP antibodies), Complement levels (C3 and C4), ds-DNA and plan for a kidney biopsy if suspicion for nephritis. –expert opinion
Don’t dismiss hydroxychloroquine! Its delayed onset of action (about 3 months) leads many to underestimate its benefits which include the prevention of flares, joint and skin damage, and development of organ involvement (Dorner 2019).
It’s very broad, but consider the following:
Get your radar up for lupus in patients with the following features or symptoms:
The classification criteria were recently updated by ACR and EULAR (Dorner 2019). These are meant to identify patients for enrollment in clinical trials, but not for bedside diagnosis. For example, a positive ANA is required in the 2019 lupus classification criteria, but bedside diagnosis of lupus does not require a positive ANA.
The ANA is sensitive but not specific. It’s positive in 10% of healthy women. On the other hand, the ENa panel (see below) contains specific antibodies for various autoimmune diseases.
Order an ANA if there is a high index of suspicion for autoimmune disease (e.g. lupus or mixed connective tissue disease). This usually requires organ system involvement (e.g. painless mouth ulcers, hematuria). An ANA is less helpful when a patient has a bunch of nonspecific features (e.g. fatigue and arthralgias). —Dr. Jonas’ expert opinion
Kashlak pearl: ANA titers less than 1:80 can probably be ignored. ANA titers above 1:80 must be interpreted in clinical context i.e. a positive titer in the presence of hematuria, rash and cytopenias is very suggestive of lupus.
Kashlak pearl: A positive ANA should be followed by a thorough search of the medication list for possible drug induced lupus (hydralazine, procainamide, INH, minocycline).
Kashlak pearl: In addition to a careful history and physical exam (i.e. skin, joints, mouth), Dr. Jonas sends a CBC, TSH and urinalysis in patients with vague symptoms that might be rheumatologic.
Dr. Jonas notes that a normal CBC and urinalysis don’t rule out lupus, but they suggest there is time to follow the patient and see how their symptoms evolve.
Kashlak pearl: High suspicion for lupus: Send ANA, **ENa panel, Complement levels (C3 and C4), ds-DNA and plan for a kidney biopsy if suspicion for nephritis.
**The ENa panel varies by hospital but might contain anti-Smith, Ro/La, anti-Jo1, SCL-70, and RNP antibodies.
Dr. Jonas tailors the discussion based on the severity of symptoms.
“It’s a disease where your body makes antibodies against its own tissues…and causes inflammation in many areas including the skin, the joints, the kidneys, the brain…really any organ system…and we need to get that inflammation under control with medications and careful follow up.”Beth Jonas MD, FACR on The Curbsiders #171 Lupus in Primary Care
Note: Dr. Jonas does not recommend a specific anti-inflammatory diet as there is no evidence to support one as superior.
Dr. Jonas recommends adequate calcium, vitamin D intake and weight bearing physical activity for patients on chronic prednisone. Patients on chronic high dose steroids (>20 mg prednisone) or with a history of fractures warrant more aggressive monitoring.
Kashlak pearl: A T-score is reported for post-menopausal women undergoing bone density testing. A Z-score should be used for for children, young adults, women who are premenopausal and men under age 50 (AmericanBoneHealth.org – 2016).
Lupus itself IS NOT a contraindication to LIVE vaccines. But, LIVE vaccines should be avoided in patients on chronic high dose steroids (>20 mg prednisone), mycophenolate, cyclophosphamide or other immunosuppressive medications.
In lupus, the goal of therapy is to control the degree of inflammation.
Hydroxychloroquine takes several months (about three) to control symptoms. Thus, steroids are often given in the short term to help control symptoms. Unfortunately, hydroxychloroquine’s delayed onset of action leads many patients to underestimate it’s benefits and leads to poor adherence.
Kashlak Pearl: Don’t dismiss hydroxychloroquine! It has wide ranging benefits and disease modifying effects!
“current dogma is that all patients with SLE should be treated with hydroxychloroquine because of benefits in multiple domains (eg, improvement of rash and arthritis, reduction in risk of early cumulative damage, flare prevention, reduction in lipid concentrations, normalisation of glucose concentrations, antithrombotic and antiatherosclerotic effects, antiinfective characteristics).”Excerpt from Dorner et al 2019 https://www.ncbi.nlm.nih.gov/pubmed/31180031
Hydroxychloroquine should not be dosed more than 5 mg/kg due to risk of retinal disease. The toxicity is related to dose and duration of therapy with retinal disease found in under 1% after 5 years of therapy. Therefore, the most recent ophthalmology guidelines recommend a dilated eye exam annually after 5 years of use (Marmor 2016).
NOTE: A deeper discussion of advanced therapies for lupus was outside the scope of this podcast. Let us know if you’d like to hear a lupus 2.0 episode!
Listeners will recognize and diagnose various presentations of lupus (SLE) in primary care, counsel patients with a new diagnosis of lupus, learn the general approach to treatment, and provide preventive care.
After listening to this episode listeners will…
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Dr. Jonas reports no relevant financial disclosures. The Curbsiders report no relevant financial disclosures.
Jonas B, Williams PN, Watto MF. “#171 Lupus in Primary Care with Beth Jonas MD”. The Curbsiders Internal Medicine Podcast. http://thecurbsiders.com/episode-list September 9, 2019.
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