#171 Lupus in Primary Care with Beth Jonas MD

September 9, 2019 | By Matthew Watto, MD

Diagnose and treat lupus in primary care with tips from rheumatologist, Beth Jonas MD

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?! 

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Credits

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

Time Stamps

• 00:00 Intro, disclaimer, guest bio
• 03:03 Guest one-liner; Book recommendation*: How Doctors Think (book) by Jerome Groupman; Career advice
• 10:50 A case of hematuria and rash, Ms Luna Wolferton
• 12:00 Differential diagnosis for a patient with symptoms suggestive of lupus
• 13:50 Clues for lupus on history and exam
• 15:42 Classification criteria versus making a clinical diagnosis of lupus (Dorner 2019)
• 18:17 ANA, what is it good for?
• 21:33 Basic lab workup (CBC, urinalysis and TSH)
• 25:10 Labs to send once lupus is suspected
• 28:15 Does Ms Wolferton have lupus? 
• 29:10 Spiel for explaining lupus to a patient
• 31:20 ANA titers
• 33:02 Are there classic phenotypes of lupus?
• 34:40 Lifestyle factors
• 38:20 Bone density and chronic steroids
• 39:42 Vaccinations; Antimicrobial prophylaxis
• 41:55 Hydroxychloroquine; Treatment goals in lupus
• 44:38 Steroids and add on therapies e.g. methotrexate, Belimumab
• 47:55 The importance of hydroxychloroquine
• 49:15 Drug-induced lupus (e.g. hydralazine, isoniazid, minocycline, procainamide)
• 51:17 Take Home Points; Plug: Thurston Arthritis Center at UNC Chapel Hill
• 53:40 Outro

Clinical Pearls – Lupus in Primary Care 

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).

Dr. Jonas’ Take Home Points

1. A positive ANA does not make the diagnosis of lupus.
2. Patients with lupus must be followed very closely, AND counseled on the importance of adherence to treatment and monitoring.
3. Caring for lupus is a team effort.

Diagnosis of Lupus in Primary Care

Differential diagnosis

It’s very broad, but consider the following:

• Other rheumatologic diseases
• Non-autoimmune rheumatologic diseases (i.e. fibromyalgia)
• Infections
• Malignancy (e.g. hematologic)
• Endocrine disorders (e.g. thyroid disease)

Clues from the history and exam that suggest Lupus

Get your radar up for lupus in patients with the following features or symptoms:

• Younger age
• African American race
• Painless mouth ulcers (as opposed to apthous ulcers which are painful)
• Arthralgias in the hands
• Pregnancy complications
• Rash
• Chest pain/pericardial symptoms
• Hematuria
• Chronic symptoms

Lupus classification criteria

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 Dreaded 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).

Initial lab workup if suspicion is low

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. 

Lab workup if suspicion is high or initial testing positive

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.

The Spiel: Explaining lupus to your patient

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

Lupus in Primary Care

Lifestyle factors in the care of lupus should include:

• Smoking cessation (may be an environmental trigger for autoimmunity)
• Sun protection
• Cardiovascular risk reduction 
• Physical activity as tolerated

Note: Dr. Jonas does not recommend a specific anti-inflammatory diet as there is no evidence to support one as superior. 

Who needs bone density testing? 

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).

Vaccinations for Lupus in Primary Care

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.

Hydroxychloroquine Treatment of Lupus in Primary Care

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!

Patients on hydroxychloroquine might be protected from the development of organ involvement (including renal — Guillermo 2009) and more severe disease (Fessler 2005).  According to Dorner 2019, 

“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!

Goals

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. 

Learning objectives

After listening to this episode listeners will…

1. Recognize its myriad manifestations and diagnose lupus. 
2. Perform an initial workup for suspected lupus
3. Interpret positive ANA titers and order appropriate follow up testing
4. Counsel patients with a new diagnosis of lupus
5. Educate patients on nonpharmacologic therapy for lupus
6. Discuss the basics of lupus treatment and monitoring/prevention including vaccinations, control of vascular risk factors, antimicrobial prophylaxis and promotion of bone health
7. Provide excellent primary care and preventive medicine to co-manage lupus
8. Counsel patients with a new diagnosis of lupus

Links*

1. How Doctors Think (book) by Jerome Groupman
2. Thurston Arthritis Research Center at UNC Chapel Hill
3. Dorner T Novel paradigms in systemic lupus erythematosus Lancet 2019 [ https://www.ncbi.nlm.nih.gov/pubmed/31180031 ] –Nice review of 2019 lupus classification criteria and current approaches to treatment and monitoring. 
4. Chowdhary VR Broad Concepts in the Management of Systemic Lupus Erythematosus Mayo Clin Proc 2017 [ https://www.ncbi.nlm.nih.gov/pubmed/28473038 ]-Plan language review of lupus for primary care audience. 

*The Curbsiders participates in the Amazon Services LLC Associates Program, an affiliate advertising program designed to provide a means for sites to earn advertising commissions by linking to Amazon. Simply put, if you click on my Amazon.com links and buy something we earn a (very) small commission, yet you don’t pay any extra.

Disclosures

Dr. Jonas reports no relevant financial disclosures. The Curbsiders report no relevant financial disclosures.

Citation

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.