ACR Guidelines for Vaccines in Patients with Rheumatic & Musculoskeletal Diseases 2022 Vaccine Guidance: • Expanded Indications for Specific Vaccines • Multiple Vaccines in one Day • Non-Live vs Live Vaccinations By Mithu Maheswaranathan, MD @MithuRheum #Vaccine #vaccination #Guidelines #rheumatology #management #immunocompromised

Guidelines for Perioperative Management of Antirheumatic Medication in Patients with Rheumatic Diseases Undergoing Elective Total Hip or Total Knee Arthroplasty Continue or withhold prior to Surgery • DMARDS • Biologics • JAKi By Dr. Mithu Maheswaranathan, MD @MithuRheum #rheumatology #medications #hold #perioperative #guidelines #management #autoimmune #pharmacology #table

Myositis Specific Antibodies (MSAs) Dermatomyositis: MDA5, TIF1y, NXP2, Mi-2, SAE Anti-Synthetase Syndrome: Jo-1, PL7, PL12, EJ, OJ Immune Mediated Necrotizing Myopathy (IMNM): HMGCR, SRP Inclusion Body Myositis (IBM) + others: NT5c1A/cN1A By Mithu Maheswaranathan, MD @MithuRheum #Myositis #Specific #Antibodies #MSAs #clinical #diagnosis #rheumatology #table

Scleroderma Renal Crisis (SRC) Clinical Presentation of Scleroderma Renal Crisis: 1) Acute kidney injury 2) Abrupt onset of hypertension 3) Abnormal urinalysis 3) microangiopathic hemolytic anemia (MAHA) Pathophysiology: • Precise pathogenesis is unknown but may involve vasculopathy, endothelial cell activation, ↓ perfusion • Biopsy → endothelial cell injury, arteriolar thickening, fibrinoid necrosis, fibrotic "onion-skinning" of arteries Management Pearls: • Hospitalization for BP control & Monitoring • Renal Biopsy to confirm diagnosis and rule out other pathology (e.g. pauci-immune GN) • Initiate ACEi (short-acting, e.g. captopril, to allow titration q6 hours) for initial therapy. • If uncontrolled with ACEi, add other BP meds • Refractory cases → consider PLEX (overlap TTP or aHUS), eculizumab, vasodilators (ERAs) By Mithu Maheswaranathan, MD @MithuRheum #SRC #Scleroderma #Renal #Crisis #rheumatology #nephrology #diagnosis #management

CNS Manifestations of Systemic Lupus Erythematosus (SLE) Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic Meningitis, Myelopathy, Encephalopathy, Cognitive Dysfunction, Cerebrovascular Disease, PRES Pathophysiology: Pathogenesis involves the formation of autoantibodies that will cause ischemia and/or inflammation, leading to vasculopathy, thrombosis and/or blood-brain barrier permeability Diagnosis of Lupus Cerebritis: Labs, Imaging, Lumbar Puncture, EEG Treatment: • Inflammatory Pathway - Lupus Cerebritis Induction Rx: high-dose glucocorticoids + cyclophosphamide • Ischemic Pathway - Aspirin, anticoagulants (for secondary APS), Secondary prevention (statin for CVA) By Dr. Gabriela Figueiredo Pucci @gabifpucci (@neudrawlogy) and Mithu Maheswaranathan, MD @MithuRheum (@RheumOnePagers) #Systemic #Lupus #Erythematosus #SLE #CNS #neurology #rheumatology #cerebritis #diagnosis #management #treatment

Systemic Sclerosis (Scleroderma) Multi-system autoimmune disease characterized by vasculopathy and progressive fibrosis of skin and internal organs 3 Subtypes of Scleroderma (SSc): • Limited cutaneous systemic sclerosis (LcSSc) - distal skin thickening, distal to elbows/wrists and knees • Diffuse systemic sclerosis (DcSSc; diffuse) - skin thickening extends proximally to arms, thighs, and/or trunk • Systemic Sclerosis sine scleroderma (ssSSc) - fibrosing internal organ involvement without skin thickening Signs and Symptoms: • Cardiac: myocardial fibrosis, pericarditis • Hepatic: PBC (primary biliary cholangitis) overlap • Gastrointestinal: GERD, dysmotility, SIBO, GAVE, pseudoobstruction • Cutaneous ("salt and pepper" skin changes): skin thickening, sclerodactyly, telangiectasias, poikiloderma, calcinosis • Pulmonary: ILD (interstitial lung disease), PAH (pulmonary arterial HTN) • Renal: scleroderma renal crisis (SRC) • Musculoskeletal: joint involvement, acro-osteolysis, myositis overlap • Vascular: Raynaud's, digital ulcers Mithu Maheswaranathan, MD @MithuRheum #Systemic #Sclerosis #Scleroderma #SSc #rheumatology #diagnosis #signs #symptoms #testing #workup

Scleroderma Mimics • Scleredema • Scleromyxedema (papular mucinosis) • Eosinophilic Fasciitis • Nephrogenic Systemic Fibrosis • POEMS Syndrome • Diabetic Cheiroarthropathy • Environmental Exposures By Mithu Maheswaranathan, MD @MithuRheum #Scleroderma #Mimics #rheumatology #differential #diagnosis

Lupus Nephritis Primer Class I - Minimal Mesangial Class II - Mesangial Proliferative Class Ill - Focal Proliferative Class IV - Diffuse Proliferative Class V - Membranous Class VI - Advanced Sclerosing By Mithu Maheswaranathan, MD @MithuRheu and Dr. Caitlyn Vlasschaert @DrFlashHeart #Lupus #Nephritis #SLE #Classification #Classes #differential #diagnosis #management #nephrology

Aortitis - Differential Diagnosis Framework Non-Infectious Aortitis: • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome, Relapsing polychondritis, Behcet's Disease, Polyarteritis Nodosa (PAN), ANCA associated vasculitis (rare) • Rheumatic Diseases: IgG4-Related Disease (IgG4-RD), Rheumatoid Arthritis (RA), Systemic lupus erythematosus (SLE), Sarcoidosis, HLA-B27-related spondyloarthropathies (SPA) • Other Causes: Cancer, Histiocytic disorders (Erdheim-Chester), Immune Checkpoint Inhibitors (irAEs) (rare), Idiopathic Infectious Aortitis: • Most common bacterial causes include: Staphylococcus spp., Salmonella spp., Streptococcus pneumoniae • Mycobacterial Causes: M.tuberculosis, M. bovis • Fungi: Candida, Cryptococcus, Aspergillus, Histoplasma, Paracoccidioides • Viruses: HIV, VZV By Mithu Maheswaranathan, MD @MithuRheu and Dr. Sara Dong @swinndong #Aortitis #Differential #Diagnosis #rheumatology

Rheumatoid Arthritis Summary Overview: chronic, inflammatory symmetric polyarthritis involving small joints of hands/feet, knees, shoulders Signs & Symptoms: • early AM stiffness, >1 hour • sx improve with activity (inflammatory) • joint pain / swelling Physical Exam • synovitis; joint swelling or tenderness • joint deformities, rheumatoid nodules, joint effusions, reduced ROM Laboratory Studies • RF (rheumatoid factor) • anti-CCP (cyclic citrullinated peptide) - ↑ specificity than RF • ESR/CRP - ↑ inflammatory marker Imaging: • XRay (erosions, joint space narrowing) • Ultrasound Diagnosis: CLINICAL DIAGNOSIS based on history, exam and imaging Treatment: • steroids (prednisone), ideally limit for acute flare • intra-articular corticosteroid injections • DMARDs: Methotrexate (MTX), Sulfasalazine (SSZ), Hydroxychloroquine (HCQ), Leflunomide (LEF) • JAKi / Small Molecules: Tofacitinib, Baracitinib, Upadacitinib • Biologics: Anti-TNF (Adalimumab, Etanercept, Infliximab, Certolizumab, Golimumab), IL-6 (tocilizumab), Abatacept By Mithu Maheswaranathan, MD @MithuRheum via @RheumOnePagers #Rheumatoid #Arthritis #diagnosis #management #rheumatology