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Rheumatology High Value Care Recommendations The American College of Physicians, in collaboration with o All tumor necrosis factor inhibitors provide similar bene- multiple other organizations, is engaged in a worldwide fit in patients with rheumatoid arthritis (see Item 36). initiative to promote the practice of High Value Care (HVC). . Laboratory testing is usually not necessary to diagnose The goals of the HVC initiative are to improve health osteoarthritis but is helpful if other causes of arthritis are care outcomes by providing care ofproven benefit and being considered or to help define the safety ofpotential reducing costs by avoiding unnecessary and even harmful therapies. interventions. The initiative comprises several programs . Topical NSAIDs are safe and effective for treatment of that integrate the important concept ofhealth care value knee and hand osteoarthritis and should be considered (balancing clinical benefit with costs and harms) for a before oral NSAIDs (see Item 24). given intervention into a broad range of educational mate- . The use of chondroitin sulfate (with or without glucos- rials to address the needs oftrainees, practicing physicians, amine), biologics, intra-articular hyaluronic acid, platelet- and patients. rich plasma, stem cells, or botulinum toxin is not recom, mended in the treatment of knee osteoarthritis HVC content has been integrated into MKSAP 19 in several (see Item 5o). important ways. MKSAP 19 includes HVC-identified key o Acetaminophen provides no benefit for hip or knee points in the text, HVC-focused multiple-choice questions, osteoarthritis; it may be considered as add-on therapy and, in MKSAP Digital, an HVC custom quiz. From the text for short-term and episodic use but not as initial therapy and questions, we have generated the following list of HVC (see Item 54). recommendations that meet the definition below of high o Tai chi is as beneficial as physical therapy for knee and hip value care and bring us closer to our goal of improving osteoarthritis pain (see Item 34). patient outcomes while conserving finite resources. . Exercise is beneficial in hip, knee, and hand osteoarthri- HighValue Care Recommendation: A recommendation to tis; no one exercise program is superior (see Item A2). choose diagnostic and management strategies for patients in o Arthroscopic surgery is not indicated in patients with specific clinical situations that balance clinical benefit with osteoarthritis unless there is joint buckling, instability, or cost and harms with the goal of improving patient outcomes. locking, or a concomitant and symptomatic mechanical disorder. Below are the High Value Care Recommendations for the o Patients with fibromyalgia should not be treated with Rheumatology section of MKSAP 19. anti-inflammatory drugs, including NSAIDs and glu- o An accurate history and a thorough musculoskeletal cocorticoids, and do not respond to opioids, with the physical examination are essential to diagnose and differ- exception of tramadol (see Item 7). entiate inflammatory and noninflammatory symptoms o Borrelia burgdorferi DNA can be detected by polymerase and can help to avoid unnecessary testing. chain reaction in slnovial fluid, but this test offers no o Antinuclear antibody testing should not be performed in advantage to serologic testing (see Item 12). a patient with nonspecific symptoms and normal find- o A muscle biopsy is not necessary to diagnose dermato- ings on clinical examination because it does not establish myositis in patients with characteristic clinical and labo- the diagnosis of a connective tissue disease. ratory findings (see Item 38). r Antinuclear antibody (ANA) subserologr testing should . A muscle biopsy can help to confirm the diagnosis of not be performed routinely, even in the setting of a pos- inclusion body myositis but is not needed when clinical itive ANA result, without strong clinical suspicion of an features are characteristic (see Item 45). underlying connective tissue disease. o In the absence of additional suggestive symptoms, physi- . Radiography is usually the first imaging test ordered in cal findings, and supporting laboratory data, oral dryness the evaluation of rheumatologic diseases because it is should not be attributed to a rheumatologic condition readily available, is inexpensive, exposes patients to only (see Item 47). a low level of ionizing radiation, and is useful in monitor- o If a patient has many features of spondyloarthritis, a pos- ing arthritis progression. itive HLA-B27 result adds little to the posttest probability . Ultrasonography is an inexpensive means to assess (see Item 5). soft-tissue abnormalities, assess disease activity, and o Antibiotics generally are not indicated in reactive arthritis assist with tendon or joint injections. because they do not affect illness outcomes (see ltem 67).

Rheumatology High Value Care Recommendations The American College of Physicians, in collaboration with o All tumor necrosis factor inhibitors provide similar bene- multiple other organizations, is engaged in a worldwide fit in patients with rheumatoid arthritis (see Item 36). initiative to promote the practice of High Value Care (HVC). . Laboratory testing is usually not necessary to diagnose The goals of the HVC initiative are to improve health osteoarthritis but is helpful if other causes of arthritis are care outcomes by providing care ofproven benefit and being considered or to help define the safety ofpotential reducing costs by avoiding unnecessary and even harmful therapies. interventions. The initiative comprises several programs . Topical NSAIDs are safe and effective for treatment of that integrate the important concept ofhealth care value knee and hand osteoarthritis and should be considered (balancing clinical benefit with costs and harms) for a before oral NSAIDs (see Item 24). given intervention into a broad range of educational mate- . The use of chondroitin sulfate (with or without glucos- rials to address the needs oftrainees, practicing physicians, amine), biologics, intra-articular hyaluronic acid, platelet- and patients. rich plasma, stem cells, or botulinum toxin is not recom, mended in the treatment of knee osteoarthritis HVC content has been integrated into MKSAP 19 in several (see Item 5o). important ways. MKSAP 19 includes HVC-identified key o Acetaminophen provides no benefit for hip or knee points in the text, HVC-focused multiple-choice questions, osteoarthritis; it may be considered as add-on therapy and, in MKSAP Digital, an HVC custom quiz. From the text for short-term and episodic use but not as initial therapy and questions, we have generated the following list of HVC (see Item 54). recommendations that meet the definition below of high o Tai chi is as beneficial as physical therapy for knee and hip value care and bring us closer to our goal of improving osteoarthritis pain (see Item 34). patient outcomes while conserving finite resources. . Exercise is beneficial in hip, knee, and hand osteoarthri- HighValue Care Recommendation: A recommendation to tis; no one exercise program is superior (see Item A2). choose diagnostic and management strategies for patients in o Arthroscopic surgery is not indicated in patients with specific clinical situations that balance clinical benefit with osteoarthritis unless there is joint buckling, instability, or cost and harms with the goal of improving patient outcomes. locking, or a concomitant and symptomatic mechanical disorder. Below are the High Value Care Recommendations for the o Patients with fibromyalgia should not be treated with Rheumatology section of MKSAP 19. anti-inflammatory drugs, including NSAIDs and glu- o An accurate history and a thorough musculoskeletal cocorticoids, and do not respond to opioids, with the physical examination are essential to diagnose and differ- exception of tramadol (see Item 7). entiate inflammatory and noninflammatory symptoms o Borrelia burgdorferi DNA can be detected by polymerase and can help to avoid unnecessary testing. chain reaction in slnovial fluid, but this test offers no o Antinuclear antibody testing should not be performed in advantage to serologic testing (see Item 12). a patient with nonspecific symptoms and normal find- o A muscle biopsy is not necessary to diagnose dermato- ings on clinical examination because it does not establish myositis in patients with characteristic clinical and labo- the diagnosis of a connective tissue disease. ratory findings (see Item 38). r Antinuclear antibody (ANA) subserologr testing should . A muscle biopsy can help to confirm the diagnosis of not be performed routinely, even in the setting of a pos- inclusion body myositis but is not needed when clinical itive ANA result, without strong clinical suspicion of an features are characteristic (see Item 45). underlying connective tissue disease. o In the absence of additional suggestive symptoms, physi- . Radiography is usually the first imaging test ordered in cal findings, and supporting laboratory data, oral dryness the evaluation of rheumatologic diseases because it is should not be attributed to a rheumatologic condition readily available, is inexpensive, exposes patients to only (see Item 47). a low level of ionizing radiation, and is useful in monitor- o If a patient has many features of spondyloarthritis, a pos- ing arthritis progression. itive HLA-B27 result adds little to the posttest probability . Ultrasonography is an inexpensive means to assess (see Item 5). soft-tissue abnormalities, assess disease activity, and o Antibiotics generally are not indicated in reactive arthritis assist with tendon or joint injections. because they do not affect illness outcomes (see ltem 67). xill

Rheumatology L Approach to the Patient t(rY P0lflT5 . Inflammatory symptoms include pain, erythema, swell- i With Rheumatologic ing, and warmth; noninflammatory conditions usually Disease lack these features, except for pain. o An accurate history and a thorough musculoskeletal HVC lnflammatory Versus physical examination are essential to diagnose and dif-

Approach to the Patient t(rY P0lflT5 . Inflammatory symptoms include pain, erythema, swell- i With Rheumatologic ing, and warmth; noninflammatory conditions usually Disease lack these features, except for pain. o An accurate history and a thorough musculoskeletal HVC lnflammatory Versus physical examination are essential to diagnose and dif- L Noninflammatory Pain ferentiate inflammatory and noninflammatory symp- The differentiation between inflammatory and noninflamma toms and can help to avoid unnecessary testing. tory signs and symptoms is central to the evaluation of patients with musculoskeletal pain. Autoimmune conditions Arthritis typically present with inflammation, whereas mechanical or Monoarthritis degenerative disorders are characteristically noninflamma- Monoarthritis involves a single joint and is classified as acute tory. The cardinal signs of inflammation are pain, erythema, or chronic. swelling, and warmth; noninflammatory conditions usually Acute monoarthritis can be noninflammatory (e.g., lack these features, except for pain. Patients may simultane- caused by trauma, hemarthrosis, or internal derangement) or ously experience more than one type of pain. Table 1 com infl ammatory (e. g., crystal-induced or infectious). Evaluation pares the features of inflammatory and noninflammatory for infectious arthritis should be guided by the clinical presen- arthritis. tation and examination, but suspicion should always be high. Joint aspiration is usually the most effective means of diagnos ing the underlying cause. The M usculoskeletal Examination Chronic inflammatory monoarthritis (>26 weeks) can be An accurate history and a thorough musculoskeletal physical caused by chronic infection (e.g., mycobacterial, fungal, or examination are essential to diagnose and differentiate inflam- Borrelia burgdorferi) or by autoimmune rheumatologic dis- matory and noninflammatory symptoms and can help to ease. Synovial fluid cell count analysis can help determine the avoid unnecessary testing. Musculoskeletal pain may be presence of inflammation but may be inadequate for diagnosis; articular, periarticular, or referred. Pain with passive range assessment for systemic disease (complete history; examina- of motion suggests an articular condition, whereas pain tion; and laboratory studies, including serologic testing) may be only with active range of motion suggests a periarticular indicated. Rarely, synovial biopsy may be required to rule out condition. chronic infection, deposition diseases, or malignancy. See MKSAP 19 General Internal Medicine 1 for more Chronic noninflammatory monoarthritis is usually caused information. by osteoarthritis.

L Noninflammatory Pain ferentiate inflammatory and noninflammatory symp- The differentiation between inflammatory and noninflamma toms and can help to avoid unnecessary testing. tory signs and symptoms is central to the evaluation of patients with musculoskeletal pain. Autoimmune conditions Arthritis typically present with inflammation, whereas mechanical or Monoarthritis degenerative disorders are characteristically noninflamma- Monoarthritis involves a single joint and is classified as acute tory. The cardinal signs of inflammation are pain, erythema, or chronic. swelling, and warmth; noninflammatory conditions usually Acute monoarthritis can be noninflammatory (e.g., lack these features, except for pain. Patients may simultane- caused by trauma, hemarthrosis, or internal derangement) or ously experience more than one type of pain. Table 1 com infl ammatory (e. g., crystal-induced or infectious). Evaluation pares the features of inflammatory and noninflammatory for infectious arthritis should be guided by the clinical presen- arthritis. tation and examination, but suspicion should always be high. Joint aspiration is usually the most effective means of diagnos ing the underlying cause. The M usculoskeletal Examination Chronic inflammatory monoarthritis (>26 weeks) can be An accurate history and a thorough musculoskeletal physical caused by chronic infection (e.g., mycobacterial, fungal, or examination are essential to diagnose and differentiate inflam- Borrelia burgdorferi) or by autoimmune rheumatologic dis- matory and noninflammatory symptoms and can help to ease. Synovial fluid cell count analysis can help determine the avoid unnecessary testing. Musculoskeletal pain may be presence of inflammation but may be inadequate for diagnosis; articular, periarticular, or referred. Pain with passive range assessment for systemic disease (complete history; examina- of motion suggests an articular condition, whereas pain tion; and laboratory studies, including serologic testing) may be only with active range of motion suggests a periarticular indicated. Rarely, synovial biopsy may be required to rule out condition. chronic infection, deposition diseases, or malignancy. See MKSAP 19 General Internal Medicine 1 for more Chronic noninflammatory monoarthritis is usually caused information. by osteoarthritis. TABLE I . Features of lnflammatory Versus Noninflammatory Arthritis Feature lnflammatory Arthritis Noninf lammatory Arthritis Morning stiffness >60 min; worsens with immobility <30 min Constitutional sym ptoms Fever; fatigue; malaise Generally absent Physical examination findings Erythema; warmth; soft-tissue swelling; joint Minimal or no warmth; no soft-tissue swelling; effusions; reduced ROM is frequent bony enlargement and joint effusions may occur in osteoarthritis; reduced ROM may occur Synovialiluid Leukocyte count >2000/pL(2.0 x 10e /L), Leukocyte count of 200-2000/pL (0.2-2.0 x 1 0ell), predominantly neutrophils in acute inflammation predominantly monocytes and monocytes in chronic inflammation Other laboratory findings Elevated inflammatory markers (ESR, CRP); lnflammatory markers usually normal or anemia of inflammation minimally elevated

TABLE I . Features of lnflammatory Versus Noninflammatory Arthritis Feature lnflammatory Arthritis Noninf lammatory Arthritis Morning stiffness >60 min; worsens with immobility <30 min Constitutional sym ptoms Fever; fatigue; malaise Generally absent Physical examination findings Erythema; warmth; soft-tissue swelling; joint Minimal or no warmth; no soft-tissue swelling; effusions; reduced ROM is frequent bony enlargement and joint effusions may occur in osteoarthritis; reduced ROM may occur Synovialiluid Leukocyte count >2000/pL(2.0 x 10e /L), Leukocyte count of 200-2000/pL (0.2-2.0 x 1 0ell), predominantly neutrophils in acute inflammation predominantly monocytes and monocytes in chronic inflammation Other laboratory findings Elevated inflammatory markers (ESR, CRP); lnflammatory markers usually normal or anemia of inflammation minimally elevated CRP = C reactive protein; ESR = erythrocyte sedimentation rate; ROI\l = range of motion

Approach to the Patient With Rheumatologic Disease Oligoanhritis Extra-Articu I a r M a n ifestatio ns Oligoarthritis involves two to four joints, typically in an asym metric pattem. of Rheumatologic Disease Acute inflammatory oligoarthritis may be caused by Constitutional Symptoms gonorrhea or rheumatic fever. Chronic inflammatory oligoar Fever, morning stiffness, and fatigue occur in numerous rheur-na thritis can be caused by autoimmune conditions. such as tologic conditions. Fever is usually low grade but may be high and seronegative spondyloarthritis (e.g., psoriatic arthritis. reac- spiking in some conditions (e.g., adult onset Still disease and tive arthritis, arthritis related to inflammatory bowel disease, autoinflammatory diseases). Morning stiflhess lasting more than or ankylosing spondylitis). 60 minutes is most commonly described in RA but also occurs Chronic noninflammatory oligoarthritis is usually caused with other forms of inflammatory afthritis. Significant and even by osteoarthritis. disabling fatigue is a prominent feature of fibromyalgia.

Oligoanhritis Extra-Articu I a r M a n ifestatio ns Oligoarthritis involves two to four joints, typically in an asym metric pattem. of Rheumatologic Disease Acute inflammatory oligoarthritis may be caused by Constitutional Symptoms gonorrhea or rheumatic fever. Chronic inflammatory oligoar Fever, morning stiffness, and fatigue occur in numerous rheur-na thritis can be caused by autoimmune conditions. such as tologic conditions. Fever is usually low grade but may be high and seronegative spondyloarthritis (e.g., psoriatic arthritis. reac- spiking in some conditions (e.g., adult onset Still disease and tive arthritis, arthritis related to inflammatory bowel disease, autoinflammatory diseases). Morning stiflhess lasting more than or ankylosing spondylitis). 60 minutes is most commonly described in RA but also occurs Chronic noninflammatory oligoarthritis is usually caused with other forms of inflammatory afthritis. Significant and even by osteoarthritis. disabling fatigue is a prominent feature of fibromyalgia. Skin lnvolvement Polyarthritis Skin involvement is common in rheumatologic conditions and Polyarthritis involves five or more joints. In many cases, it may go unnoticed by the patient (Table 2). It may also be an involves the small joints of the hands and/or feet. adverse eflect of n-redications used to treat rheumatologic con Acute polyarthritis (<6 weeks in duration) can be caused ditions. including skin inf'ections secondary to immunosup by viral infections (e.g., with parvovirus 819, HIV hepatitis pressive therapy. viruses, rubella, or chikungunya virus) or may be an early manifestation of chronic (>6 weeks in duration) inflammatory polyarthritis, such as rheumatoid arthritis (RA), systemic Eye lnvolvement lupus erythematosus (SLE), or psoriatic arthritis. Eye involvement in different rheumatologic diseases usually follons fairly distinct patterns, and the location and type of involvement can help narro\{ the differential diagnosis Soft-Tissue Abnormal ities (Table 3). If not quickly recognized and treated. certain fbrms ofeye involvement can have devastating consequences, includ Common nonarticular sources of musculoskeletal symptoms ing permanent loss of vision. See MKSAP 19 General Internal are the soft tissues (tendons, ligaments. and bursae) around or 'I

Skin lnvolvement Polyarthritis Skin involvement is common in rheumatologic conditions and Polyarthritis involves five or more joints. In many cases, it may go unnoticed by the patient (Table 2). It may also be an involves the small joints of the hands and/or feet. adverse eflect of n-redications used to treat rheumatologic con Acute polyarthritis (<6 weeks in duration) can be caused ditions. including skin inf'ections secondary to immunosup by viral infections (e.g., with parvovirus 819, HIV hepatitis pressive therapy. viruses, rubella, or chikungunya virus) or may be an early manifestation of chronic (>6 weeks in duration) inflammatory polyarthritis, such as rheumatoid arthritis (RA), systemic Eye lnvolvement lupus erythematosus (SLE), or psoriatic arthritis. Eye involvement in different rheumatologic diseases usually follons fairly distinct patterns, and the location and type of involvement can help narro\{ the differential diagnosis Soft-Tissue Abnormal ities (Table 3). If not quickly recognized and treated. certain fbrms ofeye involvement can have devastating consequences, includ Common nonarticular sources of musculoskeletal symptoms ing permanent loss of vision. See MKSAP 19 General Internal are the soft tissues (tendons, ligaments. and bursae) around or 'I Medicine 2 {br additional information on ocular rnanifesta away from the joints. Isolated tendon andi'or ligament involve tions of rheumatologic disease. ment usually suggests noninflammatory disorders, such as mechanical injury/irritation, overuse, or degeneration (e.9.. lnternal Organ lnvolvement rotator cuff disorders or tennis elbow). Disorders of wide Rheumatologic diseases fiequently aft'ect internal organs. with spread musculoskeletal pain (e.g., fibromyalgia) also cause different diseases tending to follow characteristic patterns symptoms localizing to these structures. (Table 4). The enthesis is a complex structure at the site of the inser tion of a tendon or ligament onto the bone. Severe persistent I(EY POIXI inflammation of the enthesis (enthesitis) strongly suggests o Rheumatologic disease can cause constitutional symp- spondyloarthritis, especially when it affects multiple sites. The toms and extra-articular manifestations affecting the inflammation may extend along the associated tendon and skin, eyes, and internal organs. Iocal ligaments; it results in dactylitis ("sausage digits"), which is typically a feature ofspondyloarthritis, particularly psoriatic arthritis. See MKSAP 19 General Internal Medicine 1 for more Laboratory Studies information. Laboratory studies are useful for diagnosing rheumatologic dis eases. identilying the extent/severity of involvement, evaluating t(EY POlt{rs disease activity, and monitoring therapeutic responses. Because HVC . Joint aspiration is usually the most effective means of of limited specificity. results of these tests should always be diagnosing the underlying cause of acute monoarthritis. interpreted in the context of the clinical history and physical o Chronic noninflammatory arthritis is usually caused by examination and should be applied with great caution, if at all, osteoarthritis. in the setting of low pretest probability (see MKSAP 19 General r Isolated tendon and/or ligament involvement usually Internal Medicine 1 for discussion of pretest probabilities).

Medicine 2 {br additional information on ocular rnanifesta away from the joints. Isolated tendon andi'or ligament involve tions of rheumatologic disease. ment usually suggests noninflammatory disorders, such as mechanical injury/irritation, overuse, or degeneration (e.9.. lnternal Organ lnvolvement rotator cuff disorders or tennis elbow). Disorders of wide Rheumatologic diseases fiequently aft'ect internal organs. with spread musculoskeletal pain (e.g., fibromyalgia) also cause different diseases tending to follow characteristic patterns symptoms localizing to these structures. (Table 4). The enthesis is a complex structure at the site of the inser tion of a tendon or ligament onto the bone. Severe persistent I(EY POIXI inflammation of the enthesis (enthesitis) strongly suggests o Rheumatologic disease can cause constitutional symp- spondyloarthritis, especially when it affects multiple sites. The toms and extra-articular manifestations affecting the inflammation may extend along the associated tendon and skin, eyes, and internal organs. Iocal ligaments; it results in dactylitis ("sausage digits"), which is typically a feature ofspondyloarthritis, particularly psoriatic arthritis. See MKSAP 19 General Internal Medicine 1 for more Laboratory Studies information. Laboratory studies are useful for diagnosing rheumatologic dis eases. identilying the extent/severity of involvement, evaluating t(EY POlt{rs disease activity, and monitoring therapeutic responses. Because HVC . Joint aspiration is usually the most effective means of of limited specificity. results of these tests should always be diagnosing the underlying cause of acute monoarthritis. interpreted in the context of the clinical history and physical o Chronic noninflammatory arthritis is usually caused by examination and should be applied with great caution, if at all, osteoarthritis. in the setting of low pretest probability (see MKSAP 19 General r Isolated tendon and/or ligament involvement usually Internal Medicine 1 for discussion of pretest probabilities). suggests noninflammatory disorders, such as mechani- cal injury/irritation, overuse, or degeneration. Tests That Measure lnflammation o Persistent inflammation of the enthesis (enthesitis) Ery.throcyte Sedimentation Rate The erythrocyte sedimentation rate (ESR) measures the fall of strongly suggests spondyloarthritis. erythrocytes (in millimeters per hour) through anticoagulated

suggests noninflammatory disorders, such as mechani- cal injury/irritation, overuse, or degeneration. Tests That Measure lnflammation o Persistent inflammation of the enthesis (enthesitis) Ery.throcyte Sedimentation Rate The erythrocyte sedimentation rate (ESR) measures the fall of strongly suggests spondyloarthritis. erythrocytes (in millimeters per hour) through anticoagulated 2

I : I : Approach to the Patient With Rheumatologic Disease i TABLE 2. Dermatologic Manifestations of Rheumatologic Disease Rheumatologic Disease Dermatologic Manifestations Systemic lupus erythematosus Acute cutaneous lupus erythematosus (commonly a malar rash); subacute cutaneous lupus erythematosus; photosensitive rash; chronic cutaneous lupus erythematosus (most commonly discoid lupus); oral ulcerations (on the tongue/hard palate; usually painless); alopecia; lupus panniculitis (painful, indurated subcutaneous swelling with overlying erythema of the skin) Dermatomyositis Gottron papules (erythematous plaques on extensor surfaces of MCP and PIP joints); photodistributed poikiloderma, including shawl sig n (over the back and shoulders) and V sign (overthe posterior neck/back or neck/upper chest); heliotrope rash (violaceous rash on the upper eyelids); mechanic's hands (hyperkeratotic, fissured skin on the palmar and lateral aspects of fingers); nailfold capillary abnormalities; holster sign (poikiloderma along lateralthigh); can occur in the absence of myositis (a myopathic dermatomyositis) Systemic sclerosis Skin tightening, thickening, and hardening; nailfold capillary changes; calcinosis; telangiectasias; decreased oral aperture; bi- or tri-color Raynaud phenomenon Vascu litis Palpable purpura; nodules; ulcers; necrosis; Raynaud phenomenon if cryoglobulins present Behget syndrome Painful oral and genital ulcers; erythema nodosum; acne/folliculitis; pathergy (skin inflammation/ ulceration from minor trauma) Sarcoidosis Erythema nodosum; infiltrated plaques; maculopapular and papular lesions; nodules; soft infiltrates of the nose (lupus pernio); on blanching with a glass slide, sarcoid skin lesions reveal "apple jelly" discoloration Psoriatic arthritis Plaque psoriasis typically on extensor surfaces, umbilicus, glutealfold, scalp, and behind ears; pustular psoriasis on palms and soles; nail pitting; onychodystrophy Reactive arthritis Keratoderma blennorrhagicum (psoriasiform rash on soles, toes, palms); circinate balanitis (psoriasiform rash on penis) Adult-onset Still disease Evanescent, salmon-colored rash on trunk and proximal extremities Rheumatic fever (secondary to Erythema marginatum (annular pink to red nonpruritic rash with central clearing) streptococcal infection) Lyme disease Erythema chronicum migrans (slowly expanding, often annual lesion with central clearing)

TABLE 2. Dermatologic Manifestations of Rheumatologic Disease Rheumatologic Disease Dermatologic Manifestations Systemic lupus erythematosus Acute cutaneous lupus erythematosus (commonly a malar rash); subacute cutaneous lupus erythematosus; photosensitive rash; chronic cutaneous lupus erythematosus (most commonly discoid lupus); oral ulcerations (on the tongue/hard palate; usually painless); alopecia; lupus panniculitis (painful, indurated subcutaneous swelling with overlying erythema of the skin) Dermatomyositis Gottron papules (erythematous plaques on extensor surfaces of MCP and PIP joints); photodistributed poikiloderma, including shawl sig n (over the back and shoulders) and V sign (overthe posterior neck/back or neck/upper chest); heliotrope rash (violaceous rash on the upper eyelids); mechanic's hands (hyperkeratotic, fissured skin on the palmar and lateral aspects of fingers); nailfold capillary abnormalities; holster sign (poikiloderma along lateralthigh); can occur in the absence of myositis (a myopathic dermatomyositis) Systemic sclerosis Skin tightening, thickening, and hardening; nailfold capillary changes; calcinosis; telangiectasias; decreased oral aperture; bi- or tri-color Raynaud phenomenon Vascu litis Palpable purpura; nodules; ulcers; necrosis; Raynaud phenomenon if cryoglobulins present Behget syndrome Painful oral and genital ulcers; erythema nodosum; acne/folliculitis; pathergy (skin inflammation/ ulceration from minor trauma) Sarcoidosis Erythema nodosum; infiltrated plaques; maculopapular and papular lesions; nodules; soft infiltrates of the nose (lupus pernio); on blanching with a glass slide, sarcoid skin lesions reveal "apple jelly" discoloration Psoriatic arthritis Plaque psoriasis typically on extensor surfaces, umbilicus, glutealfold, scalp, and behind ears; pustular psoriasis on palms and soles; nail pitting; onychodystrophy Reactive arthritis Keratoderma blennorrhagicum (psoriasiform rash on soles, toes, palms); circinate balanitis (psoriasiform rash on penis) Adult-onset Still disease Evanescent, salmon-colored rash on trunk and proximal extremities Rheumatic fever (secondary to Erythema marginatum (annular pink to red nonpruritic rash with central clearing) streptococcal infection) Lyme disease Erythema chronicum migrans (slowly expanding, often annual lesion with central clearing) MCP = metacarpophalangea; PIP = proximal interphalangeal.

TABLE 2. Dermatologic Manifestations of Rheumatologic Disease Rheumatologic Disease Dermatologic Manifestations Systemic lupus erythematosus Acute cutaneous lupus erythematosus (commonly a malar rash); subacute cutaneous lupus erythematosus; photosensitive rash; chronic cutaneous lupus erythematosus (most commonly discoid lupus); oral ulcerations (on the tongue/hard palate; usually painless); alopecia; lupus panniculitis (painful, indurated subcutaneous swelling with overlying erythema of the skin) Dermatomyositis Gottron papules (erythematous plaques on extensor surfaces of MCP and PIP joints); photodistributed poikiloderma, including shawl sig n (over the back and shoulders) and V sign (overthe posterior neck/back or neck/upper chest); heliotrope rash (violaceous rash on the upper eyelids); mechanic's hands (hyperkeratotic, fissured skin on the palmar and lateral aspects of fingers); nailfold capillary abnormalities; holster sign (poikiloderma along lateralthigh); can occur in the absence of myositis (a myopathic dermatomyositis) Systemic sclerosis Skin tightening, thickening, and hardening; nailfold capillary changes; calcinosis; telangiectasias; decreased oral aperture; bi- or tri-color Raynaud phenomenon Vascu litis Palpable purpura; nodules; ulcers; necrosis; Raynaud phenomenon if cryoglobulins present Behget syndrome Painful oral and genital ulcers; erythema nodosum; acne/folliculitis; pathergy (skin inflammation/ ulceration from minor trauma) Sarcoidosis Erythema nodosum; infiltrated plaques; maculopapular and papular lesions; nodules; soft infiltrates of the nose (lupus pernio); on blanching with a glass slide, sarcoid skin lesions reveal "apple jelly" discoloration Psoriatic arthritis Plaque psoriasis typically on extensor surfaces, umbilicus, glutealfold, scalp, and behind ears; pustular psoriasis on palms and soles; nail pitting; onychodystrophy Reactive arthritis Keratoderma blennorrhagicum (psoriasiform rash on soles, toes, palms); circinate balanitis (psoriasiform rash on penis) Adult-onset Still disease Evanescent, salmon-colored rash on trunk and proximal extremities Rheumatic fever (secondary to Erythema marginatum (annular pink to red nonpruritic rash with central clearing) streptococcal infection) Lyme disease Erythema chronicum migrans (slowly expanding, often annual lesion with central clearing) MCP = metacarpophalangea; PIP = proximal interphalangeal. TABTE 3, Ocular Manifestations of Systemic lnflammatory Disease Systemic lnflammatory Disease Ocular Manifestations Ankylosing spondylitis, reactive arthritis, and inflammatory bowel Uveitis (inflammation of anterior and/or posterior chamber and/or disease (anterior chamber); sarcoidosis and BehEet syndrome retina); anterior uveitis symptoms include pain, redness, visual (anterior and/or posterior chamber); granulomatosis with change; posterior uveitis symptoms include painless visual change polyangiitis (posterior chamber) or floaters Rheu matoid arth ritis; spondyloarthritis; system ic vascu litis; rarely, Episcleritis; symptoms include redness, watering, and irritation but SLE no vision loss Rheumatoid arthritis; relapsing polychondritis; systemic vasculitis; Scleritis; symptoms include severe eye pain that is worse at night inflammatory bowel disease; rarely, SLE and with eye movements; redness; photophobia Systemic vasculitis; antiphospholipid syndrome; SLE Retinal ischemia; symptoms include painless vision loss or change Sjogren syndrome Keratoconjunctivitis sicca; symptoms include dry eyes Giant cell arteritis Anterior/posterior ischemic optic neuropathy; central retinal artery occlusion; symptoms include loss of vision Sarcoidosis; granulomatosis with polyangiitis; lgG4-related Proptosis/retrobulbar inf la m matory infiltrate diseases Reactive arthritis Conjunctivitis; symptoms include red eye

TABTE 3, Ocular Manifestations of Systemic lnflammatory Disease Systemic lnflammatory Disease Ocular Manifestations Ankylosing spondylitis, reactive arthritis, and inflammatory bowel Uveitis (inflammation of anterior and/or posterior chamber and/or disease (anterior chamber); sarcoidosis and BehEet syndrome retina); anterior uveitis symptoms include pain, redness, visual (anterior and/or posterior chamber); granulomatosis with change; posterior uveitis symptoms include painless visual change polyangiitis (posterior chamber) or floaters Rheu matoid arth ritis; spondyloarthritis; system ic vascu litis; rarely, Episcleritis; symptoms include redness, watering, and irritation but SLE no vision loss Rheumatoid arthritis; relapsing polychondritis; systemic vasculitis; Scleritis; symptoms include severe eye pain that is worse at night inflammatory bowel disease; rarely, SLE and with eye movements; redness; photophobia Systemic vasculitis; antiphospholipid syndrome; SLE Retinal ischemia; symptoms include painless vision loss or change Sjogren syndrome Keratoconjunctivitis sicca; symptoms include dry eyes Giant cell arteritis Anterior/posterior ischemic optic neuropathy; central retinal artery occlusion; symptoms include loss of vision Sarcoidosis; granulomatosis with polyangiitis; lgG4-related Proptosis/retrobulbar inf la m matory infiltrate diseases Reactive arthritis Conjunctivitis; symptoms include red eye SLE = systemic lupus erythematosus.

TABTE 3, Ocular Manifestations of Systemic lnflammatory Disease Systemic lnflammatory Disease Ocular Manifestations Ankylosing spondylitis, reactive arthritis, and inflammatory bowel Uveitis (inflammation of anterior and/or posterior chamber and/or disease (anterior chamber); sarcoidosis and BehEet syndrome retina); anterior uveitis symptoms include pain, redness, visual (anterior and/or posterior chamber); granulomatosis with change; posterior uveitis symptoms include painless visual change polyangiitis (posterior chamber) or floaters Rheu matoid arth ritis; spondyloarthritis; system ic vascu litis; rarely, Episcleritis; symptoms include redness, watering, and irritation but SLE no vision loss Rheumatoid arthritis; relapsing polychondritis; systemic vasculitis; Scleritis; symptoms include severe eye pain that is worse at night inflammatory bowel disease; rarely, SLE and with eye movements; redness; photophobia Systemic vasculitis; antiphospholipid syndrome; SLE Retinal ischemia; symptoms include painless vision loss or change Sjogren syndrome Keratoconjunctivitis sicca; symptoms include dry eyes Giant cell arteritis Anterior/posterior ischemic optic neuropathy; central retinal artery occlusion; symptoms include loss of vision Sarcoidosis; granulomatosis with polyangiitis; lgG4-related Proptosis/retrobulbar inf la m matory infiltrate diseases Reactive arthritis Conjunctivitis; symptoms include red eye SLE = systemic lupus erythematosus. plasma. Erythrocytes tend to be negatively charged on their many rheumatologic diseases, as well as in nonrheumatologic surfaces, leading to repulsion and a prolonged ESR. Fibrinogen inflammatory conditions, such as chronic infections and and other acute phase reactants, as well as hypergammaglobu malignancies. The normal ESR increases with age and is usu linemia (polyclonal or monoclonal), neutralize the erythro ally higher in women. A well accepted rule of thumb is to cytes' surface charges, promoting their ability to settle at a adjust the upper limit of normal as (age in years)/2 lor men faster rate. Elevated fibrinogen levels and high ESRs are seen in and (age in years + l0) 2 for women.

plasma. Erythrocytes tend to be negatively charged on their many rheumatologic diseases, as well as in nonrheumatologic surfaces, leading to repulsion and a prolonged ESR. Fibrinogen inflammatory conditions, such as chronic infections and and other acute phase reactants, as well as hypergammaglobu malignancies. The normal ESR increases with age and is usu linemia (polyclonal or monoclonal), neutralize the erythro ally higher in women. A well accepted rule of thumb is to cytes' surface charges, promoting their ability to settle at a adjust the upper limit of normal as (age in years)/2 lor men faster rate. Elevated fibrinogen levels and high ESRs are seen in and (age in years + l0) 2 for women. 3