Hospital Medicine

Adult juvenile rheumatoid arthritis (Still's disease)

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I. What every physician needs to know.

This inflammatory disorder characterized by daily high fevers, arthritis, and an evanescent rash in children was originally described in 1896 by George Still. Since then, it has been known by various other names in adults:

  • Adult-onset Still's disease (AOSD)

  • Adult juvenile rheumatoid arthritis (adult JRA) - in the United States (US)

  • Adult juvenile chronic arthritis (JCA) - in Europe

  • Systemic onset juvenile idiopathic arthritis (soJIA) - according to the most recent classification from the International League of Associations for Rheumatology (ILAR)

The etiology is unknown. The current hypotheses include infectious triggers like viral pathogens and bacteria like Yersinia enterocolitica or Mycoplasma pneumoniae as well as genetic factors that trigger an immune response. This causes activation of neutrophils and macrophages through a complex immune cascade. There are signs of an innate immune activation involving elevated levels of the pro-inflammatory cytokines IL-1 and IL -18 in neutrophils and macrophages leading to toll like receptor activations in T cells. In addition, there are signs of an adaptive immune response in Th-1 T cells secreting IL-2. Despite the triggers no antigen has been found. Ultimately, AOSD is an inflammatory disorder involving both aspects of innate immunity and adaptive immunity.

There are 3 patterns of natural history of adult JRA: Monocyclic adult JRA with self-limited single episode that resolves within months, polycyclic with multiple flares with systemic or joint symptoms lasting weeks to years, chronic adult JRA with persistently active immune response with polyarthritis.

Severe complications can occur in up to a third of patients including Macrophage Activating syndrome (MAS).

II. Diagnostic Confirmation: Are you sure your patient has adult juvenile rheumatoid arthritis?

Adult JRA is a diagnosis of exclusion and its protean symptoms as well as its rarity make diagnosis difficult. There are multiple classification systems the most widely used being the Yamaguchi criteria and Fautrel criteria. Yamaguchi’s criteria is the most sensitive (96.2%) while Fautrel’s is the most specific (98.5%).

A. History Part I: Pattern Recognition:

The classic presentation of adult JRA is daily high fevers accompanied by an evanescent salmon colored rash on the trunk along with myalgias and polyarticular arthritis.

Adult JRA has multiple organ involvement and pattern recognition is the key to including this diagnosis in the differential. The main clinical features are as follows:

Fever

The fever of adult JRA is often quotidian or double quotidian (two fever spikes in 1 day). The temperature spikes are high with return to normal or below baseline temperature in between in 80% of the patients. It precedes most other symptoms, and so often the presentation is as a fever of unknown origin (FUO).

Rash

The diagnostic rash (in 60-80% of cases) is a non-pruritic faint salmon colored macular or morbilliform rash (Figure 1) which occurs with the fever spikes. It is transient and often missed. It predominantly involves the trunk and extremities, though it may also involve the palms, soles and face. Look for the rash in areas subject to pressure like the beltline and under the breasts or it can be elicited by stroking the skin (Koebner's phenomenon). The rash is frequently misdiagnosed as a drug reaction.

Figure 1.

Salmon rash of Adult JRA

Arthritis

Arthralgias, arthritis and myositis are universal features of adult JRA though often are preceded by fever by a few weeks. Initially the arthritis is mild, transient and oligoarticular though over time it evolves into a more severe, destructive polyarthritis. It involves both small and large joints, most commonly knees, wrists, ankles, elbows and proximal interphalangeal joints (PIP) joints. Fusion of the wrists is a characteristic late finding.

Adults with JRA present with severe and debilitating myalgias, which coincide with fever spikes. However, there is no muscle weakness.

Pharyngitis

A severe, non-suppurative pharyngitis is common in adult JRA (about 70% of the cases). Relapsing episodes of fever with pharyngitis are a common presentation of this disorder.

Lymphadenopathy and hepatosplenomegaly

Slightly tender and enlarged cervical lymph nodes are seen in 50% of the patients. Mild splenomegaly (in 30-65% of cases) and/or hepatomegaly (in 30% of cases) may also be present. Given the fever, leukocytosis and lymphadenopathy, an important diagnostic differential is lymphoma.

Liver disease

Patients present with vague abdominal pain either from the liver disease or from serositis. The liver enzymes are modestly elevated in most patients and patients may develop fulminant liver failure as part of the hemophagocytic complication of adult JRA.

Serositis

Serosal inflammation in the form of pericarditis or pleural effusions is seen in a third of patients. Other cardiopulmonary manifestations include transient pulmonary infiltrates presenting as complaints of a dry cough, pleuritic chest pain and mild dyspnea. Rarely, adult JRA could be complicated by myocarditis causing arrhythmias and heart failure or life threatening pericardial tamponade.

Hematological manifestation

Adult JRA can be associated with many hematological abnormalities including but not limited to red cell aplasia, microangiopathic hemolytic anemia (MAHA), thrombotic thrombocytopenic purpura (TTP) and disseminated intravascular coagulation (DIC). However the most severe complication that is not to be missed is MAS. MAS is also known as reactive hemophagocytic syndrome (RHS) or hemophagocytic lymphohistiocytosis (HLH).

MAS is characterized by persistent high fever, pancytopenia, abnormal liver function, encephalopathy, DIC, and a low erythrocyte sedimentation rate (ESR). A late but sensitive diagnostic finding is active phagocytosis by macrophages and histiocytes in the bone marrow.

A number of triggers have been implicated in the cause of MAS in adult JRA, the most important being after the onset of treatment of adult JRA with non-steroidal anti-inflammatory drugs (NSAIDs). Other triggers include viral infections such as mononucleosis, post-vaccination, a prolonged inflammatory course of untreated adult JRA, and rarely the disease modifying drugs like sulfasalazine, methotrexate (MTX) and the anti-tumor necrosis factor (TNF) biologic drugs like etanercept.

It is important to note that MAS can occur at any time during the course of adult JRA, including as the presenting symptoms. Flares of adult JRA are clinically indistinguishable from the development of MAS. However, some features such as pleuritis, adult respiratory distress syndrome (ARDS), and pancytopenia are more common in MAS.

B. History Part 2: Prevalence:

The annual incidence of adult JRA is estimated to be about 0.16 cases per 100,000 persons with equal distribution between the sexes. There is a bimodal age distribution with one peak between 15 and 25 years and the second between the ages of 36 and 46 years, though cases have been described in patients as old as 70. Amongst the juvenile idiopathic arthritides, 10-15% fall under the systemic onset subtype.

Genetic factors such as human leukocyte antigen (HLA) B17, B18, B35, DRB1, DR2 and DR5 have been shown to be associated with adult JRA in some studies.

Unfortunately, there are currently no identified factors that can predict which individuals will have an increased risk of either developing adult JRA or its complications.

C. History Part 3: Competing diagnoses that can mimic adult juvenile rheumatoid arthritis

Adult JRA is a diagnosis of exclusion. Given it often presents as fever of unknown origin the differential list is large:

  • Infectious and post-infectious etiologies like septic arthritis, Lyme disease, acute rheumatic fever, post-streptococcal arthritis, Yersinia and Mycoplasma.

  • Hematological and neoplastic disease like leukemia or lymphoma.

  • Connective tissue disorders like lupus and dermatomyositis.

  • Inflammatory conditions like sarcoidosis and periodic fever syndromes, inflammatory bowel disease and Kawasaki syndrome.

  • Drug reactions like drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome.

Differentials and distinguishing characteristics

Sepsis tends to have a hectic non-remitting fever pattern. In comparison adult JRA has a high daily spike with return to normal temperature baseline

Acute rheumatic fever involves similar organ systems but with characteristically distinguishable features. Rheumatic fever shows quick and dramatic response to salicylates, asymmetric migratory extremely painful arthritis, erythema marginatum rash, and anti-streptolysin O (ASO) and antideoxyribonuclease (antiDNAse) B antibodies (Figure 2). In comparison, adult JRA takes a few weeks to have a response to salicylates, has symmetric mild but persistent arthritis, salmon colored faint rash and may have a slight positive ASO as a non-specific inflammatory marker but not the specific Streptococcus antibodies like anti-DNAse B. Another differentiating feature is that the cardiac involvement in rheumatic fever is usually myocarditis or valvular dysfunction. However, in adult JRA it is usually pericarditis.

Figure 2.

Erythema marginatum in rheumatic fever

Post-streptococcal arthritis in comparison can appear very similar to adult JRA. It has symmetric non-migratory axial skeletal arthritis. The response to NSAIDs is slow and it has a positive ASO titer. The differentiating features are no other organ involvement, no rash, and a positive anti-DNAse B titer.

Leukemia/lymphoma patients also have fever, rash and generalized lymphadenopathy. The differentiating features are the low-grade fever, severe bone and joint pain mainly at night, and abnormal cell counts though leaning towards pancytopenia. In contrast, adult JRA has high spiking fevers, mild arthritis with morning stiffness, and leukocytosis as well as thrombocytosis. However, given that the systemic steroids used for treatment of adult JRA can delay the diagnosis and worsen the prognosis of hematological malignancies, the bone marrow biopsy is almost always conducted.

DRESS syndrome has an extremely distinctive skin eruption associated with systemic signs after prolonged use of certain drugs (Figure 3). Typically DRESS syndrome presents with a generalized erythrodermal rash, facial edema, high fever and serious visceral involvement including hepatitis, interstitial pneumonia, interstitial nephritis and myocarditis. In contrast, adult JRA has the faint salmon rash, no facial involvement, and multi-organ involvement, however, rarely renal and usually pericarditis and not myocarditis. Hematological abnormalities pathognomonically include eosinophilia in DRESS as well as lymphocytosis, while in adult JRA it is mainly a granulocyte predominant condition.

Figure 3.

Erythrodermal rash of DRESS syndrome

D. Physical Examination Findings.

The characteristic adult JRA rash is a non-pruritic, non-blanchable, faint, salmon colored rash which is macular mainly, though sometimes consists of macules and papules. It is transient and best seen during a febrile episode so re-examination during temperature spikes is helpful. Look in the truncal area and upper extremities and also in the areas that are compressed such as the belt-line and under the breasts. Sometimes stroking of the skin can elicit the rash (Koebner's phenomenon).

Carefully examine for lymph nodes, most commonly cervical lymph nodes, and hepatosplenomegaly, which is usually mildly tender. Throat exam shows non-suppurative pharyngitis.

Joint inflammation signs are mild with decreased range of motion being the more prominent feature than warmth or tenderness. The usual joints involved are of the axial and appendicular skeleton though a classic late finding is bilateral wrist fusion.

Look for pleurisy and dullness to percussion for pleural effusions and the pericardial rub of pericarditis. The patient may also have non-specific abdominal tenderness to palpation, secondary to the serositis seen in adult JRA.

Look for signs of jaundice, pallor, purpuric rash, and encephalopathy in MAS.

E. What diagnostic tests should be performed?

There are a number of laboratory findings that are characteristic for adult JRA, however none are specific for the diagnosis. It is the constellation of the clinical features above in the presence of these laboratory findings that should alert the clinician to the possibility of adult JRA.

1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

Systemic work-up to rule out etiologies especially infections mentioned in the differential above should be carried out with cultures of appropriate sites. At the minimum, basic laboratory tests including C-reactive protein (CRP) test, ESR, complete blood count (CBC) with differential, a metabolic panel including liver enzymes and liver function tests, as well as serum ferritin, glycosylated ferritin, antinuclear antibody (ANA) and rheumatoid factor (RF) should be checked.

Acute phase reactants

A marked increase in CRP and ESR is seen in almost all patients with adult JRA.

Complete blood cell count

Leukocytosis with predominant granulocytes, normocytic, normochromic anemia of chronic disease and reactive thrombocytosis are typical. Of note, presence of immature forms like bands may suggest a septic picture. Also, pancytopenia and/or schistocytes may indicate the concerning complication of MAS.

Liver tests

Elevation of transaminases and lactate dehydrogenase are common. Liver biopsy is non-specific and may range from minimally abnormal to fulminant hepatic necrosis.

Serum ferritin

Adult JRA is associated with marked increased in serum ferritin with 50% greater than 1000 nanogram/milliliter (ng/ml) and 32% greater than 1500 ng/ml (normal 40-200 ng/ml). Serum ferritin is used as a prognostic marker and elevations correlate with the disease activity. Glycosylated ferritin has a high specificity of 93% for adult JRA as it is low (less than 20%) during exacerbations. This is due to the large amount of serum ferritin overwhelming liver glycosylation. This finding is helpful to differentiate adult JRA from other rheumatic diseases though glycosylated ferritin may not be available at all healthcare centers. However, this finding also occurs in MAS.

Immunological markers

Negative ANA and RF is a minor criterion for diagnosis. However, a low titer of either test as seen in 10% of the cases, can obscure the diagnosis.

Bone marrow biopsy

The presence of fevers, leukocytosis and non-specific lymphadenopathy often leads to examination of the bone marrow to assess for an occult infection or a myeloproliferative disorder. The biopsy is undertaken especially in young adults before administration of large doses of systemic steroids.

  • Hyperplasia of the granulocytic precursors is the most common finding.

  • The diagnostic hallmark of MAS is the presence of numerous well-differentiated macrophages (histiocytes) that are engaged in active phagocytosis of hematopoietic elements.

Joint aspiration

The synovial fluid is usually inflammatory with the leukocyte count usually in the 10,000 cells/µl range, much less than the septic or acute inflammatory range. It is mainly useful to rule out septic joint in the initial oligoarthritic stage.

Other biopsies

Other than the bone marrow biopsy in MAS, biopsies in adult JRA are usually non-specific but important to rule out other disorders:

  • Skin biopsy of the rash: The findings include dermal edema and mild perivascular inflammation in the superficial dermis, consisting of lymphocytes and histiocytes. The immunofluorescence (IF) shows some vascular complement component 3 (C3) deposition. The cutaneous histopathology of adult JRA is non-specific but useful to differentiate this disorder from vasculitis, Sweet's syndrome and other neutrophilic dermatosis.

  • Lymph node biopsy shows intense, paracortical immunoblastic hyperplasia, which is distinct from other autoimmune conditions like rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and Kikuchi's disease, but similar to lymphoma. However, the immunohistochemistry in adult JRA reveals a benign, polyclonal B cell hyperplasia, which distinguishes it from lymphoma.

2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

The classic radiographic finding of adult JRA is a non-erosive narrowing of the carpometacarpal and the intercarpal joint spaces of the wrist, which often progresses to bony ankylosis causing the typical presentation of fusion of wrist joints.

Other less commonly involved joints include cervical spine, tarsal joints and distal interphalangeal (DIP) joints in the hands.

An echocardiogram should be done to evaluate for pericardial effusion.

F. Over-utilized or “wasted” diagnostic tests associated with this diagnosis.

Other biopsies including synovial, muscle and liver are non-specific and show chronic inflammatory changes, vascular engorgement and a mononuclear cell infiltrate.

Serum cytokine levels of IL-6, IL-18, TNFα, and IFNγ are frequently seen. However, these tests are often not available for routine clinical use nor are they specific for the disease and should not be performed.

III. Default Management.

Given this is a diagnosis of exclusion and of low prevalence, a rheumatological consultation should be obtained as soon as the diagnosis is being considered.

With the understanding that adult JRA can be a chronic illness that has the potential for significant morbidity, the pharmacological treatment of the articular and extra-articular manifestations has become more aggressive and initiated earlier in the course of the disease.

As a general approach, therapeutic decisions are based upon the extent and severity of organ system involvement. NSAIDs are the first line of treatment of mild disease. However, the recommendation is to move quickly to glucocorticoids followed by biological agents if the patient’s presentation is consistent with moderate or severe disease or the symptoms of mild disease do not improve. In sicker patients, systemic glucocorticoids should be initiated at the time of presentation with the biologic agents added if the disease is refractory. Disease-modifying antirheumatic drugs (DMARDs) like MTX are often added as an adjunct to the biological agents.

A. Immediate management.

NSAIDs are used for the initial treatment of mild disease. Response is not prompt as seen in rheumatic fever but rather over several days.

  • Ibuprofen 800 milligrams (mg) 4 times daily or naproxen 500 mg twice daily are the common selections.

  • There is a concern that NSAIDs can potentially trigger the MAS in patients with adult JRA. Thus close monitoring of the patient's laboratory markers should be done, especially early in the course of the disease and its treatment.

Steroids are eventually required for most patients. In fact, patients with systemic disease characterized by high fevers, debilitating joint symptoms, or internal organ involvement should receive glucocorticoids from the initial presentation.

  • The usual dose of prednisone is 0.5 to 1 milligram/kilogram (mg/kg) per day.

  • Pulse methylprednisolone at 1 gram (gm) per day for 3 days is used in life-threatening disease presentations like cardiac tamponade, fulminant liver failure, DIC, or MAS.

  • Response to steroids may be rapid, within hours to days, and two thirds of patients respond to use of steroids alone or to steroids used after a trial of NSAIDs.

B. Physical Examination Tips to Guide Management.

The hospitalist should monitor daily for resolution or worsening of inflammatory signs such as serositis, hepatosplenomegaly, lymphadenopathy, and arthritis.

Careful attention should be given for development of serious symptoms such as hypotension, purpura, altered mentation with jaundice and hypoglycemia, and multi-organ failure suggestive of cardiac tamponade, DIC, fulminant hepatic failure, and MAS respectively.

C. Laboratory Tests to Monitor Response To, and Adjustments in, Management.

To monitor response to therapy

  • Acute phase reactants especially serum ferritin and glycosylated ferritin if available.

  • Transaminases levels for improvement in liver disease.

  • Blood cell count for improvement in leukocytosis and thrombocytosis.

To monitor for side effects/complications of the therapy

  • NSAIDs: Monitor liver enzymes for transaminitis, creatinine for nephritis, stool hemoccult for gastritis and skin bullae formations for pseudoporphyria.

  • Steroids and biologic agents: Testing for latent tuberculosis with purified protein derivative (PPD) or quantiferon gold and chronic hepatitis B or C is essential prior to starting treatment DMARD and biologics as well as patients on long term prednisone. Monitoring for signs of opportunistic infections once on treatment and aggressively starting antibiotics if necessary.

To monitor for macrophage activating syndrome

  • CBC with differential to monitor for leukocytosis and thrombocytosis.

  • Transaminase level to look for worsening liver disease.

D. Long-term management.

The long-term management is dependent on the pattern of clinical course. Three main patterns have been identified for adult JRA:

Monophasic

The course lasts less than 1 year with complete resolution of the symptoms.

Intermittent

One or more disease flares with complete remission between episodes. The subsequent flares tend to be less severe and of shorter duration.

Chronic

The disease course is persistently active and usually associated with a destructive arthritis often needing total joint arthroplasty. The predictors of a chronic pattern are:

  • Early development of polyarthritis

  • Involvement of shoulders or hips

  • Need for more than 2 years of systemic glucocorticoids

Biological agents are used for long-term anti-inflammatory effects and to allow removal of or decrease in the glucocorticoid doses.

TNF-alpha inhibitors like etanercept, infliximab and adalimumab have been shown to be useful in small case series.

  • Etanercept shows modest improvement after 6 months of treatment.

  • Infliximab shows resolution of symptoms of fever, rash, arthralgia, and hepatosplenomegaly soon after even the first infusion, and even some patients refractory to glucocorticoids and MTX showed improvement.

  • In spite of response to these agents, the long-term efficacy is questionable given that most patients discontinue treatment due to either loss of efficacy or the occurrence of adverse effects.

Anakinra, a recombinant human IL-1 receptor antagonist was used in patients with adult JRA who are refractory to glucocorticoids and TNF inhibitors after 2 to 4 weeks of treatment. Clinical improvement within 3 days and improvement in laboratory markers soon after was seen. Given its efficacy, it is being considered for second line therapy in patients with systemic adult JRA that does not respond to steroids. Its down-sides are its short half-life requiring daily injections and local site reactions which can cause patients to discontinue treatment. Further studies with IL-1 antagonists with longer half-lives are currently underway.

Rituximab, a chimeric monoclonal antibody, which decreases peripheral B-lymphocytes over 6 to 12 months, is being used for adult JRA refractory to other biological agents.

DMARDs are used in adjunct to other agents due to inadequate response or the development of side effects with other agents. These are not used as a single or first line agent.

  • MTX is often used as a glucocorticoid-sparing agent.

  • Cyclosporine is used in cases of refractory adult JRA or in patients with MAS.

E. Common Pitfalls and Side-Effects of Management

It is important to exclude other possible etiologies from the spectrum of clinical features, especially treatable causes like infections.

Though this is a relatively uncommon disease and serious complications are rare, delay in diagnosis of MAS can be life-threatening.

IV. Management with Co-Morbidities

N/A

A. Renal Insufficiency.

Avoid NSAIDS in patients with significant renal impairment. Doses of methotrexate may need be reduced by 50% in patients with creatinine clearance (CrCl) < 50 milliliters/minute (mL/min). Refer to individual medication for dosing in patients with CrCl <30 ml/min.

B. Liver Insufficiency.

Evaluate further to rule out it is not a progression of the disease severity. Otherwise, no change in standard management.

C. Systolic and Diastolic Heart Failure

No change in standard management.

D. Coronary Artery Disease or Peripheral Vascular Disease

No change in standard management.

E. Diabetes or other Endocrine issues

Glucocorticoids can worsen or induce hyperglycemia thus strict control of blood sugars is required.

F. Malignancy

No change in standard management.

G. Immunosuppression (HIV, chronic steroids, etc.).

Biologic agents can reactivate latent tuberculosis (TB), thus TB testing before starting biologic agents is strongly recommended.

H. Primary Lung Disease (COPD, Asthma, ILD)

No change in standard management.

I. Gastrointestinal or Nutrition Issues

No change in standard management.

J. Hematologic or Coagulation Issues

Evaluate further to rule out it is not a progression of the disease severity. Otherwise, no change in standard management.

K. Dementia or Psychiatric Illness/Treatment

No change in standard management.

V. Transitions of Care

A. Sign-out considerations While Hospitalized.

Examination of patient during a temperature spike to look for the evanescent salmon coloured rash if the diagnosis is still being considered.

Careful attention should be given to the development of serious symptoms and signs such as hypotension, purpura, altered mentation with jaundice and hypoglycemia, and multi-organ failure suggestive of cardiac tamponade, DIC, fulminant hepatic failure, or MAS.

B. Anticipated Length of Stay.

A few days usually unless presenting with a serious complication which can take weeks to resolve.

C. When is the Patient Ready for Discharge.

Once there is a clear trend towards resolution of symptoms, especially any organ system involvement, and the patient has transitioned to oral medication.

D. Arranging for Clinic Follow-up

1. When should clinic follow up be arranged and with whom.

The patient should be seen within the week and every few weeks in the early course to monitor for side effects, relapses and treatment side effects. The patient should be followed by a rheumatologist, physical therapist and general internist.

2. What tests should be conducted prior to discharge to enable best clinic first visit.

Basic laboratory tests including CRP, ESR, CBC with differential, a metabolic panel including renal and liver function tests, as well as acute phase reactants including serum ferritin.

3. What tests should be ordered as an outpatient prior to, or on the day of, the clinic visit.

Cell count and complete metabolic panel.

E. Placement Considerations.

None

F. Prognosis and Patient Counseling.

The extra-articular features are self -limiting and resolve in 50% of cases within 5 years of diagnosis. Severe destructive joint disease is only present in 25% of patients. However, some patients, usually young adolescents, may develop rare, life-threatening complications like pericardial tamponade, systemic vasculitis and MAS.

The functional status of patients with adult JRA is generally good, even in the setting of a chronic disease pattern. In one series even though one third of the patients had a chronic disease pattern 90% of them had a functional class of grade 1. Also, joint arthroplasty has significantly improved the functional status of many adult JRA patients with chronic destructive arthritis.

Some patients may develop secondary amyloidosis due to the chronic inflammatory state.

Besides the prognosis, patients should be counseled about the signs for the rare but life-threatening complication of MAS. In addition, information regarding the immunosuppression caused by the treatment drugs should be given and appropriate precautions taken, including preventive measures like vaccinations prior to beginning treatment and keeping a high suspicion for opportunistic infections.

Assessment of response to treatment - American College of Rheumatology 20 response

At least a 20% improvement in:

  1. Swollen joints count

  2. Tender joints count

- And three of the following five variables:

  1. Patient assessed global disease activity

  2. Evaluator assessed global disease activity

  3. Patient pain assessment

  4. Functional disability

  5. Acute phase response

A 50% and 70% American College of Rheumatology (ACR) response relates to respective 50% (and more) or 70% (and more) improvement.

VI. Patient Safety and Quality Measures

A. Core Indicator Standards and Documentation.

Documentation of consideration and rule-out of hematological malignancies as well as trend of serum ferritin are important.

B. Appropriate Prophylaxis and Other Measures to Prevent Readmission.

Evaluation for chronic latent infections like TB, Hepatitis B and C prior to initiating treatment with systemic steroids or other immunosuppressants as well as monitoring for side effects of treatment like nephritis and gastrointestinal bleed with NSAIDs is important to prevent readmission.

VII. What's the Evidence?

Cunha, B, Lortholary, O, Cunha, C. "Fever Of Unknown Origin: A clinical Approach". The American Journal of Medicine. vol. 128. 2015. pp. 1138.e1-1138.e15.

Franchini, S. "Efficacy of Traditional and Biologic Agents in Different Clinical Phenotypes of Adult-Onset Still’s Diseas". Arthritis & Rheumatism. vol. Vol. 62. August 2010. pp. 2530-2535.

Gerfaud-Valentin, M, Jamilloux, Y, Iwaz, J, Séve, P. "Adult-onset Still's disease". Autoimmunity Reviews. vol. 13. 2014. pp. 708-722.

Lenert, A, Yao, Q. "Macrophage activation syndrome complicating adult onset Still’s disease: A single center case series and comparison with literature". Seminars in Arthritis and Rheumatism. 2015.

Mahroum, N, Mahagna, H, Amital, H. "Diagnosis and classification of adult Still’s disease". Journal of Autoimmunity. 2014. pp. 34-37.

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