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Rheumatology Connections IN THIS ISSUE Tumor Board for Immune-Related Adverse Events 3 | Psoriatic Arthritis Treatment Guidelines 5 | Hypophosphatasia 6 Inflammatory Eye Disease and Hidradenitis Suppurativa 8 | Psoriasis in a Patient with HIV-Related Kaposi Sarcoma 9 Scleredema Adultorum of Buschke 10 | Cutaneous Vasculitis 12 | Leukopenia and Lupus 14 Rheumatology Connections An Update for Physicians | Summer 2019 21581_CCFBCH_19RHE1078_ACG.indd 1 6/6/19 8:52 AM Cleveland Clinic’s Rheumatology Program is ranked among the top From the Chair of Rheumatic and Immunologic Diseases 2 in the nation in U.S. News & World Report’s “America’s Best Dear Colleagues, Hospitals” survey. I am honored to present to you the summer 2019 issue of Rheumatology Connections, an issue I think is particularly representative of the interdisciplinary nature and broad implications of the medicine we practice as rheumatologists. Rheumatology Connections, published by Cleveland Clinic’s Department of Rheumatic and Our frequent collaborations with dermatology underscore the multisystemic nature of our Immunologic Diseases, provides information specialty. Dr. Elaine Husni presents highlights of the first set of collaborative guidelines on on leading-edge diagnostic and management techniques as well as current research for psoriatic arthritis from the American College of Rheumatology and our dermatology colleagues physicians. in the National Psoriasis Foundation (p. 5). Dr. Leonard Calabrese’s ustekinumab case spanning rheumatology, dermatology and oncology is the first of its kind reported (p. 9). Dr. Soumya Please direct any correspondence to: Chatterjee offers a case study on the rare and debilitating scleredema adultorum of Buschke Abby Abelson, MD Chair, Rheumatic and Immunologic Diseases (p. 10). And Dr. Carol Langford co-authors a report on cutaneous vasculitis with a colleague in dermatology that offers important pearls for diagnostic accuracy (p. 12). Cleveland Clinic/A50 9500 Euclid Ave. Of course, our multidisciplinary collaborations do not end with dermatology. Our first article Cleveland, OH 44195 features a collaboration with Cleveland Clinic Cancer Center to effectively identify and treat 216.444.3876 patients whose cancer treatment causes immune-related adverse events (p. 3). Dr. Rula Hajj-Ali [email protected] describes her joint effort with colleagues in Cleveland Clinic’s Cole Eye Institute to explore a Managing Editor: Deborah Booth Summers relationship between inflammatory eye disease and hidradenitis suppurativa (p. 8). Dr. Emily Littlejohn and Dr. Cassandra Calabrese, the first graduate of our unique rheumatology and Graphic Designer: Kim Conard infectious disease combined residency program, offer insight into a common conundrum in our Illustration: Amanda Mendelsohn patients with systemic lupus erythematosus (p. 14). Dr. Chad Deal and I present an elderly patient living with what many consider primarily a pediatric disease (p. 6). Rheumatology Connections is written for physicians and should be relied on for medical In addition to highlighting the multisystemic, collaborative nature of the work we do, this education purposes only. It does not provide a issue illustrates our commitment to Cleveland Clinic’s tripartite mission of caring for the sick, complete overview of the topics covered and investigating their conditions and educating those who serve. We hope these stories offer you an should not replace the independent judgment of a physician about the appropriateness or risks of opportunity to connect, collaborate or consult with our team. a procedure for a given patient. Respectfully, © The Cleveland Clinic Foundation 2019 Abby Abelson, MD Cover illustration: Checkpoint inhibitor therapy. Chair, Rheumatic and Immunologic Diseases 216.444.3876 | [email protected] Page 2 | Rheumatology Connections | Summer 2019 For referrals, call 855.REFER.123 (855.733.3712) 21581_CCFBCH_19RHE1078_ACG.indd 2 5/31/19 9:45 AM A Novel Tumor Board for Patients with Immune-Related Adverse Events COLLABORATING TO TREAT THIS EMERGING COMPLICATION OF CPI By Cassandra Calabrese, DO CASE A 62-year-old male presented to my rheumatology started on prednisone 15 mg daily, and within days clinic for evaluation of joint and muscle pain in the his symptoms disappeared. He reported not having setting of checkpoint inhibitor therapy (CPI) to treat felt this good in the past two years. Prednisone his cancer. He was diagnosed with metastatic renal was decreased to 12.5 mg two weeks later without cell carcinoma in 2018. He received Gamma Knife® worsening of symptoms. therapy to a brain lesion in early July 2018, then Regarding his cancer treatment, after four cycles of Dr. Calabrese (calabrc@ started combination CPI with ipilimumab (anti- combination therapy he was switched to monthly ccf.org; 216.445.6996) CTLA-4) and nivolumab (anti-PD-1). monotherapy with nivolumab, with plans to continue is associate staff in the Department After his first infusion, he felt stirrings of pain in his for the foreseeable future. After subsequent infusion, of Rheumatic and he had transient recurrence of joint pain and hands involving the fingers and wrists. After the Immunologic Diseases, second infusion, this pain significantly worsened, and stiffness which have since resolved. He continues on holds a joint appointment he developed swelling of the proximal interphalangeal prednisone 7.5 mg daily. At this point, I presented in the Department of joints, metacarpophalangeal joints and wrists. The the patient’s case at our immune-related adverse Infectious Diseases, elbows and shoulders were also painful with severe event (irAE) monthly tumor board to discuss next and is board-certified in rheumatology and stiffness, to the point that he had difficulty performing steps in management, because although his tumor infectious diseases. his activities of daily living. was responding to CPI, the treatment was causing a debilitating rheumatic irAE. Prior to CPI, he had knee pain from advanced right knee osteoarthritis, but no other history of This case highlights many important issues rheumatic or autoimmune disease. At presentation, surrounding patients who develop rheumatic irAEs his fingers and wrists were swollen. He was unable from CPI: What is the optimal treatment? Will the to make a fist, lift his shoulders or walk due to irAE persist? Is it safe to be on prednisone long term pain and stiffness. The laboratory workup revealed while receiving CPI? Our multidisciplinary tumor an erythrocyte sedimentation rate of 46 mm/hr, board was created to help answer challenging a C-reactive protein level of 2.4 mg/dL, negative questions such as those generated by this case. rheumatoid factor and anti-CCP antibodies. He was The immune-related adverse events tumor board tumor board in September 2017. The goal of the tumor In 2015, the Department of Rheumatic and board is to discuss new and/or challenging cases of Immunologic Diseases started seeing an influx of irAEs, review the extant literature and receive input on patients with rheumatic irAEs from CPI. At the time, very interprofessional management. The field of irAEs related little was known about irAEs in general, and by 2016 we to checkpoint inhibitor therapy is growing quickly, and had established an interdisciplinary referral clinic with multidisciplinary collaboration is crucial for patient care, oncology to help manage these patients. as well as to advance our knowledge in the field. The number of patients increased, as did the uncertainty A typical tumor board surrounding diagnosis and proper management of irAEs. Cleveland Clinic Cancer Center hosts the monthly This prompted creation of a multidisciplinary monthly meeting. In the beginning, mostly oncologists and Visit clevelandclinic.org/rheum Rheumatology Connections | Summer 2019 | Page 3 21581_CCFBCH_19RHE1078_ACG.indd 3 6/6/19 8:54 AM Figure. A) CTLA-4 is expressed on the T-cell surface and binds CD80/86 on the antigen-presenting cell to downregulate the early stages of T-cell activation. B) Blocking CTLA-4 with ipilimumab, an immune checkpoint inhibitor, enhances T-cell responses against the tumor. C) PD-1 binds to its ligand, PD-L1, in the periphery to downregulate the immune response. D) Blockade of PD-1 or PD-L1 by various checkpoint inhibitors reinvigorates the immune response. rheumatologists attended. Now we have regular attendees from colleagues and I presented the impact of these novel irAE tumor gastroenterology, ophthalmology, cardiology and pulmonology, boards on clinician confidence in managing irAEs. We surveyed past depending on the cases presented. The week before the meeting, a board participants on the educational value of the meetings as well as coordinator from oncology emails the group asking for cases. Anywhere their perceived impact on awareness and confidence in management from three to six cases are presented per meeting, often accompanied of irAEs. We found that two-thirds of providers felt far more aware of by a discussion of the pertinent literature. and confident in diagnosing and managing irAEs. The cases presented are about equally split between interesting cases Anecdotally, we’ve all been surprised by how much everyone that we learn from in hindsight and cases with real-time decisions to enjoys and looks forward to this monthly meeting. For many of us, make. For the latter, the gathering of multiple specialties has helped it’s our favorite conference, perhaps because it’s an emerging and significantly in brainstorming and presenting data from the literature or changing field,
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