Features of hemolysis include fever, chills, anemia, elevated bilirubin in serum and urine, low serum haptoglobin and reticulocytosis. \200 Acute thrombocytopenia i s a relatively frequent presentation. \200 If severe, both hemolytic anemia (Hb < 7 mg/dL ) and thrombocytopenia (platelets < 25,000) require high dose prednisone 60 \200\22380 mg/day and intravenous immunoglobulin therapy. Pediatric SLE \200\224acute nephritis \200 The most common lesion is diffuse proliferativ e GN (30 \200\22345% cases). \200 One-third have hypertension, which may need aggressive management. \200 All have microscopic hematuria and proteinuria > 500 mg/24 hours. Howeve r, up to one-third may have a normal serum albumin, and about 50% maintain a normal GFR. Prognosis and therapy of nephritis is guided by the active ISN-grade pathological lesion an d chronicity index; thus biopsy is important. \200 Management includes high dose steroids and cyclophosphamide 750 mg/m 2 IV monthly for 6 \200\22312 months, then quarterly IV cyclophosphamide or mycoph enolate mofetil, especially in older children (see Figure 22.3). In acute fulminant renal disease consider plasmapheresis, although this is not routinely done. Pediatric SLE \200\224acute hematological manifestations \200 Overt hemolysis occurs in < 10%, thrombocytopenia in 15 \200\22345%. \200 Bleeding is uncommon. \200 Most with thro mbocytopenia respond to steroids. IVIG can be used; rituximab may be bene \201 cial in ref ractory cases, although this has not been well studied. A high index of suspicion is needed to diagnose catastrophic APLS. It is characterized by m ultiple organ thromboses and microangiopathic changes. All cases require working closely with hematologists as highly informed interpretation of detailed serial coagulation studies is required. \200 The treatment of hematologic manifestations of SLE is shown in Figure 22.4. 5 7 2 C H A P T E R Diffuse proliferative Focal proliferative with necrotizing lesions Membranous Mesangial focal proliferative Mild or no proliferation Significant proliferation 2 2 R h e u m a t o l o g i c Prednisone 2 mg/kg/day in divided doses (maximum 60 mg/day) Prednisone 1 mg/kg/day (maximum 30 mg) and Prednisone: 1 \200\2232 mg/kg depending upon degree of proliferation (maximum 30 \200\22360 mg/day) Prednisone: 2mg/kg (maximum 60 mg/day) IV Cyclophosphamide (500 \200\2231000 mg/m2) monthly \2276 doses; every 3 months \2274 dose s and every 6 months \2272 doses e m e r g e n c i e s If non-responsive Consider IV Cyclophosphamide (regime as for diffuse proliferative disease) Fig. 22.3 Therapy \202 owchart for treating pediatric SLE nephritis. Reproduced with the permission of Oxford University Press from the Oxford Textbook of Rheum atology 3e , edited by Isenberg, David et al . ACUTE SLE 573 Leucopenia Thrombocytopenia Anemia If unresponsive to treatment of systemic disease Usually marker of disease activity If persistent consider If unresponsive to treatment of systemic disease other causes including drug-induced Prednisone: Prednisone: 1 mg/kg/day 1 \200\2232mg/kg/day (maximum 60 mg) Consider pulse (maximum 30 mg) IV gammaglobulin methylprednislone Consider pulse cyclophosphamide Splenectomy is very controversial Fig. 22.4 Flowchart for treating pediatric hematologic manifestations of SLE. Reproduced with the permission of Oxford University Press from the Oxford Textbook of Rheum atology 3e , edited by Isenberg, David et al . CHAPTER 22 Rheumatologic emergencies 574 Systemic vasculitis A severe vasculitis \202 are should be treated aggressively because p ermanent damage from tissue ischemia may occur rapidly. Patients diagnosed with pulmonary c apillaritis or glomerulonephritis should generally be treated with pulse steroids. The spec i \201 c management of each type of vasculitis is outlined in relevant sections of Chapter 14. Giant cell (temporal) arteritis \200 Because giant cell arteritis (GCA) can lead to crani al ischemic events including blindness and stroke, it is important to treat empirically when suspicion is high . The prevalence of giant cell arteritis increases with age. Visual changes, jaw claudication , and diplopia in the setting of B-type symptoms all support the diagnosis of giant cell ar teritis. Empiric therapy starts with prednisone 1mg/kg/d for 1 month; if the patient presents with visual complaints, it would be prudent to initiate treatment with met hylprednisolone 1 g IV qd for 3 days. Daily aspirin has been associated with a decreased risk of cranial ischemic events, and should be used as part of standard therapy unless there is clear contr aindication. Treatment should not be delayed; biopsy is diagnostically useful up to 2 weeks after steroid therapy is initiated. To optimize yield, temporal artery biopsy should be bilateral, with samples of at least 1.5 cm in length. \200\234Severe \200\235 vasculitis \200 Patients with active vasculitis can quickly deve lop manifestations that threaten life or the function of a vital organ. These patients are sometimes referred to as having \200\234severe \200\235 vasculitis. Severe manifestations of the small vessel vasculitides include pulmonary hemorrhage (or capillaritis) and glomerulonephritis. Severe manifestations of med ium vessel vasculitis include mononeuritis multiplex (i.e., foot drop/wrist drop) or mesent eric angina/ischemia. \200 When glomerulonephritis is suspected, renal biopsy can be very useful to con \201 rm the diagnosis. Severe vasculitis is generally treated with pulse solumedrol 1g IV qd for 3 days, followed by prednisone 1mg/kg/d. Most patients with severe vasculitis will also be treated with oral cyclophosphamide 1.5 \200\2232.0 mg/kg/d. Lower doses should be used in the elderly or in p atients with renal insuf \201 ciency. Cyclophosphamide places patients at risk for Pneumocystis infection, and appropriate chemoprophylaxis should be instituted. Oral cyclophosphamide should be administered in the morning in a single dose to minimize risk of hemorrhagic cystitis. In patients who are at high risk for infection, treatment with intravenous immunoglobulin or plasmapheresis may be appropriate. In patients who continue to decline despite immunosuppression, serious consideration should be given to the possibility that the patient has an infection mimicking a vasculitis \202 are. SCLERODERMA (Scl) CRISES 575 Scleroderma (Scl) crises Renal crisis \200 This may manifest as an acute or subacute hype rtensive crisis, usually within the \201 rst four years after diagnosis of diffuse scleroderma (dcSSc). It can be the presenting feature of SSc (Chapter 12). Glucocorticoids may place a patient with scleroderma at elevated risk of developing scleroderma renal crisis. An abrupt increase in BP >150/85 and new renal insuf \201 ciency are consistent with this diagnosis. Other manifestations include what would be expected with hypertensive emergency, such as microangiopathic hemolytic anemia, encephalopathy, and hype rtensive retinopathy. Acute Scl renal crisis management \200 ACE inhibitors are the cornerstone of management o f renal crisis. The patient should be treated with escalating doses of captopril until bloo d pressure is brought under control. ARB and calcium channel blockers can be added sequenti ally if captopril is inadequate. Fast drops in blood pressure should be avoided, as low perfusion pressures in abnormal rena l vessels may worsen renal failure. Consult nephrology about hemodialysis if necessary. \200 Prompt initial treatment often l eads to re-establishment of good renal function. Pulmonary hypertension \200 Primary pulmonary arterial hypertension (PAH) occurs as a complication of lcSSc, although it can also occur in dcSSc (both as a primary feature and s econdary to pulmonary \201 brosis) Echocardiography can be used to screen for PAH; an RVSP > 40 mm Hg is suggestive, but the d iagnosis must be con \201 rmed by right heart catheterization. Decompensated PAH presents with subacute onset of signs and symptoms consistent with right heart failure, including peripheral edema and dyspnea. Management of acute SSc-related primary PAH \200 Patients with rapidly decompensating hea rt failure secondary to pulmonary arterial hypertension should be treated with supplemental oxygen, diuresis, and c ontinuous IV prostacyclin. Diuretics decrease right ventricular preload, and can lead to signi \201 cant symptomatic relief. A large pulmonary embolism can also result in rapidly worsening of pulmonary arterial hyper tension, and should be considered in the appropriate setting. Management of subacute pulmonary arterial hypertension associated with SSc is discussed elsewhere. CHAPTER 22 Rheumatologic emergencies 576 Methotrexate-induced pneumonitis This is rare but it can occur in any patient given methotr exate (MTX). Reports suggest the incidence ranges from <0.5% to 7% of patients (vari- ation due to de \201 nition of condition). It is probably much rarer in children and adolescent s compared to adults. Life-threatening pneumonitis requir- ing hospital admission probably occurs in <1% patients taking MTX. It is thought that mild pneumonitis resolves on drug wit hdrawal alone. Patients at risk \200 Most patients suffering from pneumonitis do so within the \201 rs t few months of starting MTX or after a signi \201 cant dose change. In patients on stable-dose MTX, blood levels may change in the setting of progressive renal insuf \201 ciency or low levels of folate. Consider the diagnosis in all patients on MTX with acute onset of dry cough, dyspnea, heada che, and fever. The differential diagnosis lies between chest infection, acute pulmonary ed ema, or acute interstitial lung disease associated with the underlying condition. Immediate management of severe toxicity. \200 Stop MTX. \200 Optimal therapy for methot rexate-induced pneumonitis has not been well de \201 ned. Folinic acid (15 \200\22325 mg p o q6 h) may reverse methotrexate toxicity. Anecdotally, steroids accelerate recovery; in cases of severe decompensation, it is reasonable to treat with methylprednisolone 1g IV qd for 3 days, foll owed by prednisone 1mg/kg/d. Prednisone can be tapered over the subsequent 1 \200\2236 mont hs, depending on disease severity. The remainder of management should be focused on identifying alternate causes for respirato ry compromise (e.g., BAL and high resolution CT to exclude infection) and supportive care ( e.g., supplemental oxygen, consideration of transfusion if anemia is present) Most patients with methotrexate-induced lung injury will recover, but may have chronic lung damage as a result. 577 Index A abatacept, rheumatoid arthritis (RA), 255 abnormality patterns, patients with widespread pain, 178 \200\223179 abnormal vitamin D skin vasculitis in, 202 \200\223204 pelvis and hip region, 92 posterior hip and pelvis, age, risk factor for pelvic pain, 94 airways, pulmonary 99, 100f shoulder, 22 \200\22323 thoracic spine, 64 upper limbs, 62f wrist, 40, 41f manifestations, 215 allergic (hypersensitivity) vasculitis, 438 allopurinol, gout, 274, 275 alternative medicine. See complementary and alternative medicine (CAM) aneurysmal bone cyst, 551t radiographic features, 90t spinal tumors in children and adolesc ents, 551t metabolism, 455 \200\223456 Achilles tendonitis, 122 acromegaly, 223, 375t acromioclavicular joint angina, chest pain, 65t angiography, Takayasu \200\231s arteritis (TA), 422 American College of Rheumatology (ACR) \201 bromyalgia, 524, 525t, corticosteroid injection, ankle 143 527t corticosteroid injection, stressing the, 28 \200\22329 giant cell arteritis (GCA), 145 acupuncture 426t examination, 125 \200\223126 treatment of disorders, alternative medicine, 556 osteoarthritis, 269 polymyalgia rheumatica (PMR), 424, 425t 129 acute kidney injury (AKI), 216 acute mechanical back pain, rheumatoid arthritis (RA), ankylosing spondylitis (AS) 236, 237t Bath Ankylosing 538, 539t Sj gren \200\231s syndrome, Spondylitis Disease Activity Index (BASDAI), 293, 294t adhesive capsulitis (AC), 533 362, 363t diagnosis, 533 management of, 533 Wegener \200\231s granulomatosis (WG), 431t clinical features of, adolescents. See also amyloidosis, 375t, 520 \200\223523 clinical features of, 521t diagnostic procedures, 290 \200\223291 children; foot disorders in children and adolescents; juvenile idiopathic arthritis (JIA); oligoarticular pains in children and adolescents; spinal disorders in children and adolesce nts; widespread pain in children and adolescents diagnostic criteria, 291t diagnostic evaluation of, 522 292 \200\223293 familial Mediterranean fever, 522 \200\223523, 523t differentiating diffuse idiopathic skeletal hyperostosis (DISH) and, 293t treatment of, 522 anatomy bones and joints of lower leg and foot, 118 \200\223119 disease status and prognostic indicators in, 293 elbow, 34, 35f extensor expansion of epidemiology, 290 extra-articular disease in, \201 nger, 47f back pain, 546 \200\223550, 551t localized foot pain, 123t rehabilitation principles, 147 s kin vasculitis, 206 \200\223208 spinal tumors, 549 \200\223550, foot structure, 120 functional, of lower leg 291 \200\223292 treatment of, 294 \200\223295 anterior draw test, knee and foot, 121 hand, 46, 48f hip, 93 knee, 104, 105f, 106 long muscles and tendons of lower leg and foot, 119 \200\223120 stability, 112, 113f antibiotics, septic arthritis, 551t 563t adult onset Still \200\231s, gut and hepatobiliary manifestations, 227t adults. See also lo w back anti \201 brotic therapy, systemic sclerosis, 390, 391t antineutrophil cytoplasmic antibody (ANCA) lower leg and foot pain and disorders in adults disorders, 118 \200\223121 ANCA-associated lumbar and sacral localized foot pain, 123t monoarticular pains, 150 oligoarticular pains, vasculitis, 435 \200\223437 spine, 70 neck, 14, 16f neuroanatomy of lower leg and foot, 120 vasculitis, 418, 419 antinuclear antibodies 152 \200\223160 (ANA), prevalence in diseases, 184t rehabilitation principles, pelvic musculature, 92 \200\22393 146 INDEX 578 B cell therapy, rheumatoid arthritis (RA), 255 behavioral programs, low back pain, 539t Beh et \200\231s disease, 500 \200\223502 azathioprine antiphospholipid (antibody) gut and hepatobiliary side syndrome (APS), 352 catastrophic APS (CAPS), effects, 229 malignancy increase, 232 polymyositis (PM) and dermatomyositis (DM), 410 systemic lupus 358 \200\223359 classi \201 cation criteria, 352t clinical features of, 354, cardiovascular and pulmonary involvement, 501 \200\223502 355t epidemiology and pathology, 353 fetal loss, 354, 355t and systemic lupus clinical features and erythematosus (SLE), 345, 347t management, 500 \200\223502 eye disease, 500 \200\223501 gastrointestinal erythematosus (SLE), 338 B back pain. See also low back involvement, 502 gut and hepatobiliary manifestations, 228t thrombocytopenia, 354, 355t pain and disorders in adults; thoracic back and chest pain monoarticular/ thrombosis, 354, 355t, 357t treatment of, 356, 357t oligoarticular pain, 163t anti-TNF-A therapy musculoskeletal system involvement, 501 acute mechanical, 538, rheumatoid arthritis (RA), 539t neurological involvement, 254 \200\223256 systemic lupus cause of childhood, 84t children and adolescents, 502 oligoarthritis, 156, 157 renal involvement, 502 skin and mucosa erythematosus (SLE), 346 546 \200\223550, 551t chronic low back pain, aortic dissection, chest 544, 545t involvement, 500, 501t pain, 65t congenital scoliosis treatment of, 502 Beighton hypermobility rating, 476, 478t belimumab, systemic lupus erythe matosus (SLE), 345 aortic regurgitation, 211 arthritis (CS), 546 facet hypertrophy/facet joint (FJ) syndromes, 540 \200\223542 calcium oxalate arthritis, 282 rheumatic fever, 496 systemic, 316 \200\223320 herniated disc, 549 idiopathic scoliosis, 546 nerve root lesions, 540 neuromuscular scolios is benign joint hypermobility syndrome (BJHS), 180t arthrocentesis/ bursocentesis, elbow pain, 38 collagen disorder, 476, (NMS), 546 478, 479t arthrography, shoulder nontraumatic vertebral biologic agents, rheumatoid arthritis (RA), 252, 254 biomechanical/degenerative, thoracic s pine and chest wall, 66t pain, 31 articular disorders collapse, 542 postsurgical, 543, 543t Scheuermann \200\231s wrist and hand, 42t Assessment of osteochondritis, 547 Spondyloarthritis International Society (ASAS) spinal stenosis, 542 spinal tumors, 549 \200\223550, biomechanical factors, knee pain, 116 551t Birmingham Vasculitis Activity Score (BVAS), 419 comparison of seronegative spondyloarthridities, 289t spondylolisthesis, 548, 549f spondylolysis, 548 sterile discitis, 543 warning signs for sin ister pathology in, 539t bisphosphonates gut and hepatobiliary side criteria for axial effects, 229 spondyloarthritis, 286, 287t osteoporosis treatment, balneotherapy 451 \200\223452 criteria for peripheral spondyloarthritis, 286, 288t alternative medicine, 559 low back pain, 539t basic calcium phosphate bleomycin exposure, 376t Bohan and Peter \200\231s classi \201 cation autoantibodies, systemic (BCP) associated disease, 281 lupus erythematosus (SLE), 341t autoimmune diseases juvenile DM/PM, 415t polymyositis (PM) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), 293, 294t dermatomyositis (DM), 398t autoimmune rheumatic disease, 98t, 375t Bath Ankylosing Spondylitis Functional Index (BASFI), 293 bone pain, 187. See also metabolic bone diseases cause of myopathies, 176t Sj gren \200\231s syndrome, 367 vasculitis, 203t Bath Ankylosing Spondylitis Metrology Index (BASMI), 293 biopsy for widespread autologous hemopoietic stem cell transplantation (HSCT), systemic lupus erythematosus (SLE) , 346 pain, 185 sarcoidosis, 504 \200\223505 Bath Ankylosing Spondylitis Radiology Index (BASRI), 293 bone pathology back pain, 71 \200\22372 pain in hands from, 50 INDEX 579 back pain, 546 \200\223550, 551t causes of back pain in, pelvis and thigh pain, 94 widespread pain in adults, nailfold capillaries demonstrating normal, plate 20 547t 171, 174 localized foot pain, 123t pain assessment, 9 pediatric SLE \200\223 acute hematological ma nifestations, 571, 573f wrist and hand, 42t carcinoid syndrome, 375t carcinomatous myopathy, 176t carcinomatous bone scan density scanning, 311 foot disorders in children neuromyopathy, myopathy and malignancy, 230, 231t and adolescents, 139, 140 pediatric SLE \200\223 acute nephritis, 571, 572f polymyositis (PM) and hand and wrist disorders, cardiac disease 54 \200\22355 rheumatic fever, 496 systemic sclerosis, 387 low back pain, 79 neck pain, 19 pelvic, groin and thigh dermatomyositis (DM) in, 414 \200\223415 radiographic knee cardiac manifestations conduction abnormalities, pain, 101 212 abnormalities, 115t rehabilitation principles, shoulder pain, 31 \200\22332 thoracic back and chest coronary artery disease, 211 \200\223212 146 \200\223147 pain, 69 myocardium, 210 \200\223211 pericardium, 210 valvular disease, 211 rheumatoid factor widespread pain, 193 bone scintigraphy positive polyarthritis in childhood, 259 oligoarticular pains, 167 osteonecrosis of left cardiovascular complications Beh et \200\231s disease, 501 \200\223502 systemic lupus second-hand information, femoral head, plate 10 12 widespread bone pain erythematosus (SLE), 334, 344, 569, 570t septic arthritis, 564, 565f skin vasculitis, 206 \200\223208 spinal tumors, 549 \200\223550 , and weakness, plate 17 widespread pain, 184 Brighton criteria, benign joint hypermobility syndrome (BJHS), 476, 47 9t British Isles Lupus Takayasu \200\231s arteritis (TA), 422 cardiovascular system 551t spondyloarthridities in, rheumatoid arthritis 306 \200\223307 systemic lupus (RA), 240 Sj gren \200\231s syndrome, 366 erythematosus (SLE), 349 Chlamydia, 155 chlorambucil, gut and Assessment Group (BILAG), 340 brucellar arthritis, 156 bursitis, 116 carpal tunnel syndrome (CTS), 34, 220, 221, 223 injection of carpal tunnel, plate 15 hepatobiliary side effects, 229 chloroquine, gut and treatment of, 61 catastrophic C Caisson \200\231s disease, 470 calcaneal apophysitis, foot disorder, 135t antiphospholipid (antibody) syndrome (CAPS), 358 \200\223359 celiac disease, rheumatic feat ures, 225t hepatobiliary side effects, 229 chondrodysplasias, 479 chondromatosis, synovial, 519 chorea , rheumatic fever, calcitonin gut and hepatobiliary side Centers for Disease Control and Prevention (CDC), osteoporosis, 444 Chapel Hill Consensus C onference nomenclature, 418 polyarteritis nodosa, 429 chest pain. See also thoracic back an d chest pain 496 \200\223497 chronic, infantile, neurological, cutaneous, & articular syndrome (CINCA), juvenile idiopathic arthritis (JIA), 327 chronic active hepatitis effects, 229 osteoporosis treatment, 452 Paget \200\231s disease of bone, 467, 468 calcium, osteoporosis treatment, 451, 452 calcium oxalate arthritis, 282 calcium pyrophosphate dihydrate (CPPD) d isease, 278, 280 age of patient, 154 clinical presentations antinuclear antibodies non-neurologic and (ANA), 184t nonmusculoskeletal pathology, 65t rheumatic features, 226t chronic back pain. See also children. See also back pain adolescents; foot disorders in children and adolescents; oligoarticular pains in children a nd adolescents; spinal disorders in children and adolescents; widespread pain in children a nd adolescents management of, 544, 545t management of, of, 279t laboratory tests, 278, 280 management, 280 oligoarticular joint pain, in children and adolescents, 546 \200\223550, 551t 153t, 155 Campylobacter, 155 capillary loops chronic graft versus host disease, 375t chronic regional pain syndrome nailfold capillaries demonstrating abnormal, plate 21 diffuse arm and hand swelling, plate 5 INDEX 580 Cori \200\223Forbes disease, muscle pain/weakness, 192t coronary artery disease, involvemen t in rheumatic disease, 211 \200\223212 cutaneous polyarteritis chronic graft versus host disease (Cont.) nodosa, childhood, 207t epidemiology, 514 \200\223515 investigations, 515 management, 515 chronic widespread pain cutaneous vasculitis, 202 cyclophosphamide gut and hepatobiliary side corticosteroid injection effects, 229 (CWP) therapy malignancy increase, 232 polymyositis (PM) and dermatomyositis (DM), 411 systemic lupus \201 bromyalgia and, 524 \200\223526 treatment, 525 \200\223526 Churg \200\223Strauss syn drome, ankle and foot disorders, 145 background, 142 \200\223143 contraindications, 142 elbow joint and 434 \200\223435 Clinical Disease Activity Index (CDAI), 246 erythematosus (SLE), 345 periarticular elbow structures, 143 \200\223144 glenohumeral joint, 143 hand, 144 hip joint and periarticular hip lesions, 144 \200\223145 knee joint and periarticular cyclosporine club foot, 132, 133t collagen disorders gut and hepatobiliary side effects, 229 benign joint hypermobility syndrome (BJHS), 476, 478, 479t polymyositis (PM) and dermatomyositis (DM), 410 Ehlers \200\223Danlos syndrome, 475 \200\223476, 477t Marfan syndrome, lesions, 145 osteoarthritis, 269 principles of injection techniques, 143 \200\223145 subacr omial articulation and acromioclavicular joint, 143 D denosumab, osteoporosis treatment, 452 dependent lymphedema, skin thickening, 201t 474 \200\223475 osteogenesis imperfecta (OI), 474, 475t rare chondrodysplasias and storage disorders, 479 colon cancer, rheumatic disease and, 232 complementary and De Quervain \200\231s wrist, 144 corticosteroids tenosynovitis, 35, 44 dermatomyositis \201 bromyalgia, 526 polymyositis (PM) and dermatomyositis (DM), 410 cramps, lower leg, 1 21 CREST (calcinosis, Raynaud \200\231s, esophageal dysphagia, sclerodactyly, telangiectasi a), limited cutaneous, 374 Crohn \200\231s disease, rheumatic alternative medicine (CAM) gut and hepatobiliary manifestations, 227t, 228t balneotherapy, 559 dietary supplements, 558 electrical stimulation, 559 herbal remedies, 55 5 homeopathy, 557 hypnotherapy, 558 magnet therapy, 558 physical and hands-on malignancy increase, 232 myopathy and malignancy, 231t dermatomyositis (DM) autoantibodies in myositis, 406, 407t clinical features of, therapies, 556 phytotherapy, 555 popularity of, 554 rol \201 ng, 559 spiritual healing, 55 8 \200\223559 stress management/ 400 \200\223401 corticosteroids for, 410 cutaneous disease, 400 drug-induced myopathy, features, 225t cryoglobulinemia, 203t cryoglobulinemic vasculitis, 440 412 epidemiology and gut and hepatobiliary manifestations, 228t relaxation therapy, 558 diagnosis, 398, 399t extramuscular disease treatment in, 411 immunosuppressive agents, 410 \200\223411 thermotherapy, 559 crystal arthritides, computed tomography (CT) oligoarticular pains with, 155 crystal arthropathies elbow pain, 38 foot disorders in children and adolescents, 139 intravenous basic calcium phosphate (BCP) associated disease, 281 low back pain, 79, 81 neck pain, 19 oligoarticular pains, 167 pelvic, groin and thigh gammaglobulin (IVIg), 411 investigation of, 404 juvenile DM (JDM), calcium oxalate arthritis, 282 pain, 101 414 \200\223415 malignancy, 401 muscle biopsy, 404 muscle enzyme levels, calcium pyrophosphate dihydrate (CPPD) disease, 278, 279t, 280 gout and hyperuricemia, shoulder pain, 31 \200\22332 systemic lupus erythematosus (SLE), 337 404, 405t myositis, 400 prognosis in, 412 systemic manifestations, conduction abnormalities, 272 \200\223276 cardiac involvement in rheumatic disease, 212 congenital myopathies, 176t childhood disorde r, 188t CTLA4-Ig, rheumatoid arthritis (RA), 255 cutaneous disease, 401, 402t polymyositis (PM) and dermatomyositis (DM), 400 treatment of, 410 \200\223412 devil \200\231s claw, Harpagophytum procumbens, 555 congenital scoliosis (CS), 546 INDEX 581 epicondylitis Ehlers \200\223Danlos syndrome, diabetes injection in tennis elbow, collagen disorder, 475 \200\223476, 477t antinuclear antibodies plate 14 treatment, 38 (ANA), 184t elbow pain patterns of joint and tissue changes, 220t rheumatic disease and, epidemiology corticosteroid injection, ankylosing spondylitis, 143 \200\223144 290 diagnostic procedures, 38 electrophysiology, 38 examination, 36 \200\22338 functional anato my, 34, 35f locking, 35 \200\22336 needle arthrocentesis/ 220 \200\223221 antiphospholipid skin thickening, 201t dietary supplements, 558 differentiating diffuse syndrome (APS), 353 back pain, 70 chronic regional pain syndrome, 514 \200\223515 idiopathic in \202 ammatory myopathies, 398, 399t idiopathic skeletal hyperostosis (DISH), 293t olecranon bursocentesis, 38 disc disease, 71t, 72, 84t Disease Activity Score (DAS), 245 neck pain, 14 oligoarticular pain, 163 osteoarthritis, 262 \200\223263 polymyositis (PM) an d palpating lateral epicondyle of the humerus, 37 disease-modifying palpating medial humeral antirheumatic drugs (DMARDs) dermatomyositis (DM), 398, 399t epicondyle, 37 radiographs, 38 taking a history, 34 \200\22336 treatment, 38 \200\22339 visual inspection, 36 electrical stimulation, 559 electromyography, rheumatoid arthritis (RA), psoriatic arthritis, 249, 251 \200\223252, 253t systemic arthritis, 320 D-penicillamine, gut and 296 \200\223297 rheumatoid arthritis (RA), 236, 237t hepatobiliary side effects, 229 SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis), 304 polymyositis (PM) and dermatomyositis (DM), 406, 409t electrophysiology elbow pain, 38 wide spread pain, 183 drug history, widespread pain in adults, 176 \200\223177 drug-induced lupus Sj gren \200\231s syndrome, 362 skin vasculitis, 206 systemic sclerosis, 374, antinuclear antibodies (ANA), 184t drug-induced lupus 378 emergencies. See erythematosus (DILE), 342 undifferentiated rheumatologic emergencies endocrine manifestations spondyloarthritis, 303 drug-induced myopathy, polymyositis (PM) and dermatomyositis (DM), 412 erosive osteoarthritis, 264 \200\223265 acromegaly, 223 diabetes, 220 \200\223221 hyperparathyroidism, erythema elevatum diutinum drugs (EED), 441 222 \200\223223 causes of myopathies, erythema nodosum, 508, hypothyroidism, 221 \200\223222 rheumatic disease, 176t 509t childhood systemic esophageal re \202 ux, chest 220 \200\223223 lupus erythematosus (SLE), 349 gout, 274 \200\223275 gut and hepatobiliary side pain, 65t thyrotoxicosis, 223 enteric arthropathy, 302 enteric infection, rheumatic etidronate, Paget \200\231s disease of bone, 467 European League Against features, 225t enthesitis, oligoarticular pains, 163, 166 enthesitis-related arthritis juve nile idiopathic effects from, 229 Rheumatism (EULAR), 236, 237t, 258f knee pain, 117 rheumatoid arthritis (RA), European 247 \200\223256 systemic lupus Spondyloarthropathy Study Group (ESSG), 284 Ewing sarcoma arthritis (JIA), 326 erythematosus (SLE), 347t monoarticular/ oligoarticular pain, 163t vasculitis, 203t radiographic features, eosinophilic fasciitis, 232, Dupuytren \200\231s contracture, 90t 375t 53, 220 spinal tumors in children and adolescents, 551t scleroderma-like \201 brosing disorder, 375t, 394 skin thickening, 200, 201t eosinophilic granulomatosis EG with polyangi itis (EGPA), 434 \200\223435 gut and hepatobiliary manifestations, 228t spinal tumors in ch ildren and adolescents, 551t exercise, osteoporosis treatment, 451 extraglandular (systemic) E ear, nose and throat, Wegener \200\231s granulomatosis (WG), 430 disease, Sj gren \200\231s syndrome, 364 extramuscular disease, polymyositis (PM) and derm atomyositis (DM), 411 Eaton \200\223Lambert myasthenic syndrome (ELMS), myopathy and malignancy, 230, 231t eosinophilic myalgia syndrome, 376t eye disease Beh et \200\231s disease, 500 \200\223501 ectopic calci \201 cation, 464 INDEX 582 examination, 136 \200\223137 femoral anteversion, rheumatoid arthritis (RA), eye disease (Cont.) 248 \200\223249 golfer \200\231s elbow, 37 gonococcal arthritides clinical features, 487t p yogenic, 486 \200\223488 Wegener \200\231s granulomatosis retroversion and tibial torsion, 138f (WG), 432 eyes, oligoarthritis, 313 \200\223314 forefoot examination, 139 hindfoot examination, F face and ears, skin disorders, 136 \200\223137 gonococcal arthritis, 150, 153t gout imaging of foot, 139 \200\223140 laboratory tests, 140 midfoot examination, 137 pain during sports, 134 radiographs, 139 \200\223140 taking a history, 132 \200\223136 treatment, 140 forefoot disorders causes of hyperuricemia, 196 \200\223197 273t facet joint arthritis (FJ syndromes), 71t, 540 \200\223542 causing foot disorders, 122 \200\223123, 124 clinical conditions that can mimic gouty arthritis, 275t familial benign hypercalcemia (FBH), 461 familial hyperparathyroid syndromes, 462 familial hypocalciuric examination, 126 \200\223127, clinical features of, 272 crystalline arthropathy, 139 possible causes of pain, 150 hypercalcemia (FHH), 461 123 \200\223124 treatment, 129 diagnosis of, 273 management of gouty arthritis, 274 \200\223276 nodules associated with, familial hypophosphatemic foreign protein, vasculitis, rickets, 456 203t familial Mediterranean fever fractures, rheumatoid arthritis (RA), 242 Freiberg \200\231s disease, 135t, plate 16 (FMF), 522 \200\223523, 523t oligoarticular joint pain, monoarticular/ 153t, 155 risk factors, 273t oligoarticular pain, 163t 471t, 472 oligoarticular pains in graded activity programs, adults, 155 low back pain, 539t granuloma faciale (GF), 441 granulomatosis. See Wegener \200\231s granu lomatosis (WG) family history G gait patterns oligoarticular pains, 156, 165 low back pain, 74 \200\22375 spinal disorders, 87, 88 walking in bare feet, 125 gamma linol eic acid (GLA), widespread pain, 175 Fanconi syndrome, 457 febuxostat, gout, 275 Felty \200\231s syndrome, granulomatosis with polyangiitis, gut and hepatobiliary manifestations, 228t granulomatous vasculitis, childhood, 207t groin. See pelvic, groin, and 555 rheumatoid arthritis (RA), 242 feno \201 brate, gout, 276 \201 bromyalgia, 71t, 179, 180 f classi \201 cation criteria, gastrointestinal features Beh et \200\231s disease, 502 Sj gren \200\231s syndrome, 365 \200\223366 systemic lupus thigh pain gut and hepatobiliary 524, 525t erythematosus (SLE), 337t treatment, 525 \200\223526 \201 brosis, systemic sclerosis, manifestations rheumatic diseases, systemic sclerosis, 384, 378 385t 224 \200\223229, 227t, 228t rheumatological features, \201 brous dysplasia, 473 Finkelstein \200\231s test, wrist, 44 Five Factor Score (FFS), 419 \202 at feet, 132, 133t, 135t, 137 \202 exion/extension range tests, wrist, 44 genetic abnormalities, childhood, 188t genitals, systemic sclerosis 225t, 226t side effects from drugs, 229 and, 389t gut associated reactive giant cell arteritis (GCA) arthritis (GARA), 300 diagnosis of, 424, 426 \200\223427 emergency, 574 treatment of, 427 \200\223428 glandular d isease, Sj gren \200\231s syndrome, 364 glenohumeral joint gynecologic disease, 71t \202 exor carpi ulnaris (FCU), 59 \202 exor digitorum profundus (FDP), 59 H hallux valgus, foot, 124 hand. See also wrist pain \202 exor tendonopathy, 53 foot deformities, 124, 133t foot disorders in children corticosteroid injection, corticosteroid injection, and adolescents, 135t. See also lower leg and foot disorders (adults) 143 144 injection in, plate 12 movement of, 28 glucocorticoid-induced diagnoses from simple radiographs, 54t diagnostic tests, 53 \200\22355 digit deformities, 5 2 examination, 51 \200\22353 extensor expansion of background, 132 bone scan, 139, 140 common patterns of foot osteoporosis (GIO), 448, 448t glucocorticoids deformity, 133t corticosteroid injection, gut and hepatobiliary side \201 nger, 46 145 effects, 229 pregnancy, 256 functional anatomy, 46, 48f grip, 49 diagnostic procedures, 139 \200\223140 INDEX 583 corticosteroid injection, imaging, 54 \200\22355 intrinsic muscles, 46 ischemic pain, 50 \200\22351 laboratory tests, 55 long tendons, 46 muscles of hypothenar idiopathic osteoporosis, 144 \200\223145 446 \200\223447 examination, 98 \200\22399 functional anatomy, 93 testing passive hip idiopathic pulmonary \201 brosis, 376t idiopathic scoliosis, 546 IgM rheumatoid factor (R F), \202 exion and rotational movements and hip abduction, plate 9 eminence, 49t muscles of thenar eminence, 47t muscle wasting, 52 nail and \201 nger inspe ction, 244, 259 immunoglobulin, systemic lupus erythematosus (SLE), 346 immunologic features, homeopathy, 557 hormone replacement, osteoporosis treatment, 451 systemic lupus erythematosus (SLE), 337t 52 humerus, lateral epicondyle pain from bone, 50 radiographs, 53 \200\22354 stiffness, 49 \200\22350 swelling, 51, 52 tak ing a history, 49 \200\22351 tingling/pins and needles/ of, 37 immunosuppressive agents polymyositis (PM) and hydrocortisone acetate, injection therapy, 142 dermatomyositis (DM), 410 \200\223411 hydrotherapy, low back pain, 539t hydroxychloroquine systemic sclerosis, 390, numbness, 50 treatment, 55 \200\22356 trigger \201 nger, 51 weakness, 51 hands and nails, skin 391t gut and hepatobiliary side inclusion-body myositis effects, 229 pregnancy, 256 rheumatoid arthritis (RA), (IBM), 413 infection and rheumatic disease. See also rheumatic disease Lyme disease, 494, 495t management of pyogenic joint in fection, 486 \200\223487 249 \200\223250 systemic lupus disorders, 197 hands-on therapy, 556 Harpagophytum procumbens, erythematosus (SLE), 345, 347t hypercalcemia devil \200\231s claw, 555 hemarthrosis, oligoarticular management of septic causes of, 459t infancy and childhood, joint pain, 153t hematologic manifestations SLE (systemic lupus bursitis, 488 mycobacterium 459, 460t tuberculosis, 490 \200\223491 osteomyelitis, 492, 493t pathogenesis, 482, 483t pathogens as sociated with arthritis, 482, 484t parathyroid disease, 458 treatment, 459t erythematosus), 571 SLE \200\223 pediatric, 571, 573f hematopoietic involvement, hypergammaglobulinemic purpura, 440 Hypericum perforatum, systemic lupus erythematosus (SLE), 336 \200\223337 hemochromatosis, St. John \200\231s wort, 555 hypermobility, 71t, 476, 478 hyperparathyroidism, pyogenic nongonococcal and gonococcal arthritides, 486 \200\223488 rheumatic fever, 496 \200\223498 rheumatic features, 226t 222 \200\223223 hyperthyroidism, 449 hypertrophic pulmonary Henoch \200\223Sch nlein purpura, infections 228t, 439 oligoarticular pains with, hepatitis A, rheumatic features, 225t osteoarthropathy (HPOA), 232 hyperuricemia, gout and, 155 \200\223156, 164 patients taking anti- hepatitis B TNF-A drugs, 566, 567t infection with acute polyarthritis, 175t rheumatic features, 225t 272 \200\223276 hypnotherapy, 558 hypogonadism, 449 hypothyroidism, 221 \200\223222 rheumatoid arthritis (RA), 242 vasculitis, 203t hepatitis C, rheumatic features, 225t hepatobiliary disease, infectious myositis, cause of myopathies, 176t in \202 ammatory diseases childhood, 188t o ligoarticular pains, I idiopathic in \202 ammatory Sj gren \200\231s syndrome, 365 \200\223366 hepatobiliary myopathies. See also dermatomyositis (DM); polymyositis (PM) 157, 166 manifestations. See gut and hepatobiliary manifestations systemic sclerosis, 378 vasculitis, 203t in \202 iximab, ankylosing autoantibodies in myositis, 406, 407t differential diagnosis of myopathy, 406, 408t herbal remedies, 555 herniated disc, 549 hindfoot disorders spondylitis (AS), 295 injections. See steroid injections injuries, knee, 107 interleukin-6 receptor antagonists, rheumatoid arthritis (RA), 255 epidemiology and examination, 125 \200\223126, diagnosis of, 398, 399t inclusion-body myositis 136 \200\223137 possible causes of pain, (IBM), 413 122, 123t treatment, 129 necrotizing myopathy, 413 hip joint INDEX 584 cutaneous, and articular syndrome (CINCA), 327 aspiration of joint and periarticular \202 uid collections, 115 biomechanical factors, International League of Associations for Rheumatology (ILAR), 162, 259 classi \201 cation, 164t, 186 differential diagnosis 116 juvenile idiopathic of, 187t causes of anterior, 108t chronic knee arthritis (JIA), 164t, 186 juvenile psoriatic arthritis enthesitis related arthritis, 326 in \202 ammation in juvenile idiopathic arthritis, plate 18 diagnostic procedures, (JPsA), 307 oligoarthritis, 312 interstitial lung disease, fever spikes, 188, 189f general management principles, 310 \200\223311 gut and hepatobiliar y manifestations, 227t increased growth due to chronic knee inflammation, plate 18 rheumatic disease, 214 114 \200\223116 drugs, 117 examination, 110 \200\223114 joint injection therapies, intestinal by-pass surgery, rheumatic features, 225t in-toeing, 132, 133t intra- and extramedullary tumors, radiographic features, 90t 117 joint synovitis, 111 \200\223112 knee locking, 108 \200\223109 laboratory tests, 115 \200 \223116 Lachman test, 112, 113f lateral view of thigh, 107f McMurray \200\231s test, 112, juvenile psoriatic arthritis, intratendinous, 123 intravenous gammaglobulin (IVIg), polymyositis (PM) and dermatomyositis (DM), 411 intrinsic renal failure 325 oligoarthritis, 312 \200\223315 rheumatoid factor negative polyarticular JIA, 322 \200\223323 rheumatoid factor 113f magnetic resonance positive polyarticular JIA, 324 (MR), 115 active sediment, 217 bland sediment, 217 \200\223218