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Rheumatology Connections IN THIS ISSUE Uncommon Manifestations of SLE 3 | PACNS or RCVS? 6 | Psychosocial Burden of PsA 9 Advances in IL-6 Biology 10 | AHCT for Systemic Sclerosis 12 | PET in Large Vessel Vasculitis 14 Rheumatology Connections An Update for Physicians | Summer 2018 112236_CCFBCH_18RHE1077_ACG.indd 1 7/10/18 2:06 PM Dear Colleagues, Welcome to another issue of Rheumatology Con- From the Chair of Rheumatic nections. I think you’ll find our title perfectly apt as and Immunologic Diseases you browse these articles, full of connections to an array of other specialties, to our patients and to our clinical, scientific and educational missions. Dr. Chatterjee’s collaboration with the Department of Medical Oncology and Hematology for treating severe systemic sclerosis is but one example of the relevance of our field across disciplines (p. 12). Cleveland Clinic’s Rheumatology This issue also features a study of psychosocial fac- Program is ranked among the top tors in psoriatic arthritis co-authored by Dr. Husni 2 in the nation in U.S. News & and a colleague in the Neurological Institute (p. 9). World Report’s “America’s Best Our connections to neurology become even clearer Hospitals” survey. as Drs. Calabrese and Hajj-Ali offer guidance on distinguishing between a neurological condition and the more fatal, less common rheumatologic one that it mimics (p. 6). Rheumatology Connections, published by We are always searching for ways to transcend Cleveland Clinic’s Department of Rheumatic disciplinary boundaries to provide better patient care. Our multidisciplinary style of caring for patients was and Immunologic Diseases, provides information critical in a case co-authored with a colleague in pathology and Drs. Smith, Littlejohn and Zickuhr (one of on leading-edge diagnostic and management our fellows). The case demonstrates that our Lupus Clinic (p. 3) would be incomplete without our colleagues techniques as well as current research for in pathology, nephrology and dermatology, among others. physicians. Dr. Calabrese describes the impact of bench research on and the importance of basic immunology education Please direct any correspondence to: to our field in his update on advances in IL-6 biology for rheumatologists (p. 10). Dr. Langford collaborates Abby Abelson, MD Chair, Rheumatic and Immunologic Diseases with Dr. Byram, our vasculitis fellow, on an overview of using PET to assess disease activity in large vessel vasculitis (p. 14). All these articles speak to the talent and dedication of our clinicians, researchers, educa- Cleveland Clinic/A50 tors and trainees. 9500 Euclid Ave. Cleveland, OH 44195 I hope that this issue of Rheumatology Connections gives you reason to connect with our department in your 216.444.3876 work, be it in caring for the sick, investigating their conditions or educating those who serve. I look forward [email protected] to hearing your thoughts, feedback and questions. Managing Editor: Deborah Booth Summers Graphic Designer: Barbara Ludwig Coleman Respectfully,Respectfully, Illustrations: David Schumick Rheumatology Connections is written for physicians and should be relied on for medical education purposes only. It does not provide a Abby Abelson, MD complete overview of the topics covered and should not replace the independent judgment Chair, Rheumatic and Immunologic Diseases of a physician about the appropriateness or risks 216.444.3876 | [email protected] of a procedure for a given patient. © The Cleveland Clinic Foundation 2018 On the cover: Figure 1 from p. 4. Page 2 | Rheumatology Connections | Summer 2018 For referrals, call 855.REFER.123 (855.733.3712) 112236_CCFBCH_18RHE1077_ACG.indd 2 7/10/18 2:06 PM COLLAPSING GLOMERULOPATHY, EXTENSOR TENDON RUPTURES AND AUTOIMMUNE ILD Uncommon manifestations of SLE: A case report By Lisa Zickuhr, MD, Leal C. Herlitz, MD, Emily Littlejohn, DO, MPH, and Howard R. Smith, MD he Lupus Clinic, part of Cleveland Clinic’s Dr. Zickuhr Department of Rheumatic and Immu- During evolution ([email protected]) is nologic Diseases, unites specialists in MANIFESTATION At onset no. (%) T no. (%) a fellow in the Depart- Cleveland Clinic’s Rheumatology rheumatology, nephrology and dermatology to ment of Rheumatic and Program is ranked among the top care for patients with systemic lupus erythema- Immunologic Diseases. Malar rash 401 (40) 579 (58) 2 in the nation in U.S. News & tosus (SLE) and overlap syndromes. Arthritis and World Report’s “America’s Best malar rash are the most common presentations Discoid lesions 63 (6) 104 (10) Hospitals” survey. of SLE, but other organ systems can be involved, sometimes in surprising ways (Table). We share the story of a patient who came to our Lupus Subacute cutaneous lesions 27 (3) 56 (6) Clinic with uncommon manifestations of SLE. Figure. Good HDL (left). The HDL complex Photosensitivity 294is (29) made up of a lipid core, 453phospholipids (45) and A 49-year-old African American man presented protein constituents. Enzymes in the PON-1 family of atheroprotective enzymes bind to Rheumatology Connections, published by with a history of abnormal liver function tests Dr. Herlitz Oral ulcers 108the (11) major lipoprotein component238 (24) of HDL, Cleveland Clinic’s Department of Rheumatic and inflammatory joint pain. Physical exam ([email protected]; apolipoprotein A-I (ApoA-I), and are linked and Immunologic Diseases, provides information confirmed swollen and tender metacarpopha- 216.445.7605) is on leading-edge diagnostic and management Arthritis 689to (69) the antioxidant and atheroprotective840 (84) func- langeal joints, wrists and elbows and revealed Cleveland Clinic’s techniques as well as current research for tions of HDL. Dysfunctional HDL (right). An Director of Medical physicians. cervical lymphadenopathy. Laboratory investiga- increase in the oxidized ApoA-I content of Kidney Pathology in tion demonstrated the presence of anti-nuclear, Serositis 172HDL (17) causes it to lose its normal364 (36)beneficial the Department of Please direct any correspondence to: Smith, RNP, SS-A, chromatin and double- properties and acquire pro-atherogenic and Laboratory Medicine Abby Abelson, MD Nephropathy 160pro-inflammatory (16) properties.393 Absolute (39) HDL stranded DNA antibodies with elevated inflam- and Pathology. Chair, Rheumatic and Immunologic Diseases levels do not change as a result of this oxida- matory markers. Urine studies identified protein- tive stress; rather, HDL becomes dysfunc- uria. Renal biopsy showed membranous lupus Neurologic involvement 117 (12) 268 (27) Cleveland Clinic/A50 tional. This can be detected by a decrease in PON-1 enzymatic activity. (MPO = myelo- 9500 Euclid Ave. nephritis, confirming the suspected diagnoses peroxidase) Cleveland, OH 44195 of SLE and lupus nephritis. Thrombocytopenia 94 (9) 220 (22) 216.444.3876 Physicians recommended treatment with [email protected] Hemolytic anemia 38 (4) 82 (8) hydroxychloroquine, high-dose glucocorticoids Managing Editor: Deborah Booth Summers and mycophenolate, but the patient was lost Fever 361 (36) 524 (52) to follow-up. A few weeks later, he came to Graphic Designer: Barbara Ludwig Coleman Dr. Littlejohn the hospital with acute renal failure requiring ([email protected]; Illustrations: David Schumick This is the first study investigating PON-1 dialysis. Repeat kidney biopsy showed collaps- Raynaud phenomenon 184 (18) 339 (34) 216.299.9676) is activity levels in psoriatic diseases. If the ing glomerulopathy (CG), a podocytopathy, associate staff in the Rheumatology Connections is written for relationships demonstrated here can be Department of Rheu- physicians and should be relied on for medical superimposed on membranous lupus nephritis Livedo reticularis 47 (5) 137 (14) validated longitudinally, this biomarker of matic and Immunologic education purposes only. It does not provide a (Figures 1-3). Treatment with pulse steroids oxidative stress may serve as an additive Diseases. complete overview of the topics covered and and cyclophosphamide was begun, and he was Thrombosis 42 (4) 137 (14) should not replace the independent judgment CV risk stratification tool in systemic rheu- transitioned to mycophenolate upon completion of a physician about the appropriateness or risks matic diseases and may help identify sub- of cyclophosphamide induction. Renal function Myositis 38 (4) 86 (9) of a procedure for a given patient. sets of patients in need of more aggressive recovered, proteinuria improved and liver func- preventive cardiology strategies. The © The Cleveland Clinic Foundation 2018 tion tests normalized. Unfortunately, his renal relatively young age of onset for psoriatic Lung involvement 29 (3) 73 (7) disease relapsed about 18 months later. Tacro- On the cover: Figure 1 from p. 4. diseases underscores the need for further limus was added to his regimen, with clinical investigation of oxidative biomarkers that Chorea 9 (1) 16 (2) improvement. may provide an incremental benefit in Dr. Smith Ourlongitudinal patient’s SLE CV wasrisk assessment.not confined to joint Sicca syndrome 47 (5) 161 (16) ([email protected]; and renal manifestations. He also developed 216.444.4555) is Lymphadenopathy 70 (7) 119 (12) extensor tendon ruptures in his hands and right Director of the Lupus patella, thought to be from SLE and glucocor- Clinic in the Depart- ticoid use (Figure 4). About a year after his Table. Clinical manifestations at time of onset and during evolution of disease in 1,000 systemic ment of Rheumatic and lupus erythematosus patients. Republished with permission from Cervera et al.6 Immunologic
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