2025 – PAGE 423 – RHEUMATOLOGY

(DOUBLE TAKE) NEONATAL LUPUS

The baby does NOT have lupus. Neonatal lupus occurs in children of mothers with SLE due to fetal exposure to maternal SLE-related antibodies. It is rare. Findings may include increased LFTs, petechiae, rash, scaling, thrombocytopenia, third degree AV heart block with bradycardia, or hydrops fetalis (fluid accumulation in two or more fetal compartments usually due to heart failure). Diagnose by sending maternal Anti-Ro or anti-La antibodies (AKA anti-SS-A or SS-B).

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DRUG INDUCED LUPUS

Certain medications can cause an SLE type of syndrome referred to as drug induced lupus. The more common culprits include anti-seizure medications, sulfa drugs, lithium, hydralazine, and quinidine.

PEARL: If you stop the medication, this is a REVERSIBLE condition.

JUVENILE ANKYLOSING SPONDYLITIS

Juvenile ankylosing spondylitis is a chronic inflammatory condition that involves fusion of the spine (called “bamboo spine”) and inflammation of the hips. Males are more often affected. Inflammation often involves the sacroiliac joint. Pain is worse with rest, so the patient will have pain at night and morning stiffness. Then pain improves with activity or exercise. It is a seronegative spondyloarthropathy (labs are normal, ANA is negative, and ESR is either negative or only slightly elevated). Patients can also have eye findings of iritis and uveitis. Treat with NSAIDS, sulfasalazine, or steroids.

REACTIVE ARTHRITIS (AKA JUVENILE REITER SYNDROME)

Reactive arthritis (AKA Juvenile Reiter syndrome) is a seronegative spondyloarthropathy (negative rheumatologic markers) that includes symptoms of iritis (look for pain and trouble with vision), urethritis, and arthritis.

PEARL: This is often preceded by an infection! Look for a recent infectious picture due to enteric bugs in young children (Salmonella, Shigella, Yersinia) and Chlamydia in older/adolescent children (nongonococcal urethritis).

MNEMONIC: Can’t SEE, can’t PEE, and can’t CLIMB A TREE. Refers to iritis or uveitis, urethritis, and arthritis.

BEHCET SYNDROME (AKA BEHCET’S DISEASE, BEHÇET SYNDROME, ETC.)

Behcet Syndrome (AKA Behcet’s Disease) results in aphthous ulcers, genital ulcers, uveitis, arthritis, and gastrointestinal symptoms. Labs are usually negative.

PEARL: This could be confused with ulcers due to HSV. If that option is presented look again for any gastrointestinal complaints to rule in Behcet.

PSORIATIC JUVENILE IDIOPATHIC ARTHRITIS (psJIA)

Psoriatic juvenile idiopathic arthritis (psJIA) occurs in younger children (< 6 years old) who are frequently female and ANA+. They tend to have dactylitis, arthritis of the wrists, hands and feet. There is a more even ratio of male-to-female cases in older childrend. Older children may have enthesitis (pain at insertion points of ligaments and tendons), spinal disease or sacroiliac disease. Any child with psJIA can have uveitis. About 50% of children have psoriasis, which is usually mild. For the boards, you will not be tested on the consensus criteria for diagnosis, but you might be given a child with some of the above findings and mildly elevated inflammatory markers (ESR, CRP, platelets) and a slightly elevated ANA titer. The goal of treatment is to ultimately have normal labs, normal imaging and to avoid having any bony or cartilaginous damage. For mild cases, NSAIDS may be enough. For those with widespread disease, NSAIDS plus DMARDS can help to induce remission. If the patient has psoriasis, treat the psoriasis like any other patient with psoriasis.