Intriguing Patient Cases Presented at the ACR Annual Meeting Thieves Market

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SAN DIEGO—At the 2017 Thieves Market, held Nov. 6 at the ACR/ARHP Annual Meeting, rheumatologists from around the world presented patient cases to an audience of colleagues, who then voted via text messaging to choose the cases they felt were most perplexing or intriguing. The winner received a free 2018 Annual Meeting registration, and the runner-up snagged a free Meet the Professor session registration, also for the 2018 Annual Meeting in Chicago in October.

First Prize: Very Rare Extra-Pancreatic Syndrome

Marc Plier, MD, a rheumatologist at Clinique University Saint-Luc in Brussels, Belgium, won top honors for his case of a man who presented to the emergency department after four weeks of severe, widespread pain and swelling. The patient’s painful, swollen digits resembled dactylitis, and he also had a left knee effusion, fever and tender subcutaneous nodules that suggested panniculitis. Aspiration of the swelling on his hands showed a creamy, yellow fluid that looked like liquefied fat, said Dr. Plier. Knee aspiration revealed that the patient had a great deal of fluid in the joint, but no crystals. He had very high C-reactive protein levels and slightly elevated uric acid, but other laboratory test results were normal. Because of his severe pain, Dr. Plier and his colleagues ordered an arthroscopy, which showed unusual bits of free-floating fat in his joint. A synovial biopsy showed a lesion that indicated fat necrosis.

To diagnose what processes caused the patient’s bone, skin and joint manifestations, physicians measured his serum and synovial fluid lipase levels and found they were highly elevated. An abdominal CT scan showed a large duodenal mass, and the patient was diagnosed with primary pancreatic-type acinar cell carcinoma, which led to another diagnosis of PPP syndrome (i.e., pancreatitis, panniculitis and polyarthritis syndrome). This extremely rare condition has been reported in only about 60 cases worldwide, and half the time it’s related to an underlying pancreatic tumor, said Dr. Plier.

Runner-Up: Blurred Vision, Rash, Muscle Weakness

Tamara Dahhan, DO, a rheumatology fellow at Olive View-UCLA Medical Center in Sylmar, Calif., took runner-up honors with her case of a 50-year-old man with acute-onset, right upper-extremity weakness and numbness that had persisted over the previous 12 hours. For a week prior to coming to the hospital, the patient had experienced blurry vision in his right eye that was painless and progressive. He also had a red, nonpruritic rash over the bilateral lower extremities that had lasted for four months. Originally from Mexico, the patient had been living in the U.S. for 20 years and had visited his hometown about six months before coming to the hospital.

This patient had recently experienced intermittent fevers, malaise and myalgias, as well as swelling in his left knee and one testicle; both conditions had spontaneously resolved. A HEENT examination showed a significant right inferior nasal quadrant visual field defect and signs of papillitis. He had reduced muscle strength in his right upper extremity and increased sensation to light touch over the fourth and fifth digits of his right hand. He also had an elevated white blood cell count and immunoglobulin index. An autoimmune workup was negative for antinuclear antibody (ANA), even when tested twice, as well as anti-Smith and anti-RNP antibodies. He showed higher than normal levels of anti-ds-DNA antibody.

He was initially presumed to have ANA-negative systemic lupus erythematosus, but further lab testing was positive for exposure to the bacterium Brucella melitensis, leading to a diagnosis of central nervous system-related vasculitis secondary to neuro­brucellosis. He had eaten unpasteurized cheese while visiting Mexico, causing brucellosis that may have led to a false-positive ds-DNA test result. Fever, arthritis, orchitis, petechial rash and papillitis are all features of systemic brucellosis infection.

Unusual Myalgias & More

Other cases at this year’s Thieves Market may not have won the top prizes, but elicited audience applause nonetheless. They included:

Red Rice-Related Myopathy

Ariane Barbacki, MD, a resident at McGill University/Montreal General Hospital in Quebec, Canada, shared the case of a 57-year-old woman who presented to the hospital with diffuse myalgia, generalized weakness and dysphagia so severe she had to eat pureed food. She said she hadn’t taken any medications or nutritional supplements. A highly elevated creatine kinase of 9,653 stood out. Testing for myositis autoantibodies and a muscle biopsy confirmed a diagnosis of anti-HMGCR myopathy, which prompted her doctor to ask her about her diet. This health-conscious woman had consumed large amounts of camargue red rice, a food marketed as a natural way to lower cholesterol. This rice, high in plant sterols, can mimic statins in the blood and trigger myositis.

Strange Breast Lesions

Nadine Mbuyi, MD, a fellow at Brown University Alpert Medical School in Providence, R.I., shared “The Case of the Red Herring,” the story of a 48-year-old woman who presented to the hospital with painful, bilateral breast lesions with underlying edema. On one breast, the patient had a large, necrotic area surrounded by petechiae and purpura, and her right breast was firm and tender. Formerly obese, she had recently lost 200 lbs. She had been diagnosed with hepatitis C virus, but hadn’t yet undergone treatment. She had been taking calcium, vitamin D and vitamin B-12 supplements, but no anticoagulants. Her most notable laboratory test result was a positive cryoglobulinemia.

A breast biopsy showed signs of thrombo­genic vasculopathy. After a mastectomy, her pathology report showed gangrene and ischemic necrosis with severe calcification of the arterial wall, which led to her ultimate diagnosis of non­uremic calciphylaxis, a rare condition caused by calcium deposition in small blood vessels that supply the skin and cutaneous fat. Her supplements may have contributed to her lesion development.

Uncommon Myositis

Evan Mulvihill, MD, FAAP, a pediatric rheumatologist at Nationwide Children’s Hospital in Columbus, Ohio, shared the case of a 12-year-old girl admitted to the hospital after six weeks of progressive abdominal pain, mouth sores and a genital ulcer. Her systemic symptoms included fever, weight loss and fatigue, but no joint pain or swelling. She had a bump-like rash on, and tenderness and limited range of motion in, her fingers. She had decreased muscle endurance and could not keep her arm or leg raised off the bed for more than two minutes. She had also had recent bouts of shortness of breath. Suspecting connective tissue disease, the doctor gave her a myositis panel. She tested positive for the anti-MDA-5 antibody, which led to a diagnosis of anti-MDA-5-associated juvenile dermatomyositis.

Castleman’s Disease

Shivani Garg, MD, a rheumatologist at the University of Wisconsin School of Medicine and Public Health in Madison, Wisc., described her patient, a 44-year-old, African-American man who had experienced persistent, high-grade fevers for roughly six months. He also complained of abdominal pain, night sweats and unintended weight loss. His examinations suggested he had some type of generalized lymphadenopathy, and his laboratory tests showed very high erythrocyte sedimentation rate and elevated immunoglobulins. He also showed differential retroperitoneal masses. He was eventually diagnosed with multicentric Castleman’s disease, a rare lymph node disease. After rituximab treatment, this patient fared well, she said.

Susan Bernstein is a freelance journalist based in Atlanta.