30-33 Flashcards
- Reactive arthritis related to infections. Septic arthritis.
- General
- Etiology
-Reactive arthritis is the most common form of arteritis in childhood, characterised by transient joint swelling, lasting less than 6 weeks, and most commonly affecting the ankles or knees.
-It follows some form of extra articular infection, generally enteric (Salmonella, Shigella, Campylobacter, Yersinia) in young children,
in adolescence, more commonly it is the result of viral STIs in adolescents.
—Reactive arthritis is an acute spondyloarthropathy that often seems precipitated by an infection, usually genitourinary or gastrointestinal. Common manifestations include asymmetric arthritis of variable severity that tends to affect the lower extremities with sausage-shaped deformities of fingers or toes or both, constitutional symptoms, enthesitis, tendinitis, and mucocutaneous ulcers, including hyperkeratotic or crusted vesicular lesions (keratoderma blennorrhagicum). Diagnosis is clinical. Treatment involves nonsteroidal anti-inflammatory drugs and sometimes sulfasalazine or immunosuppressants.
-Reactive arthritis is associated with HLA-B27 gene, upon the triggering of the immune system, due to an error on the HLA gene, a as of yet not understood autoimmune reciton occursion, causing attack to the ureters, joints and eyes. The synovial fluid cultures are negative for any form of bacteria.
- Reactive arthritis related to infections. Septic arthritis.
- Clinical presentation
- Treatment
-Clinical presentation: include
Reactive arthritis presents with a triad of symptoms, conjunctivitis/uveitis, urethritis (men)/cervicitis (women) and of course, inflammation of the joint. This combination of symptoms was known as Reiter’s syndrome,
The classical presentation is that of urinary symptoms first (such as dysuria and frequency) followed by monoarthritis, often of the keen or sacroiliac joint. Later ocular involvement may develop if there has been no treatment. In some cases, nodules and mucocutaneous skin lesions can develop.
-Treatment is generally supportive, ABs if there is still an infection present, otherwise Analgesics, steroids and immunosuppressants may be given. The condition is self limiting and patients generally make a full recovery, however complications can occur including heart disease, amyloidosis, ankylosing spondylitis, aortitis and aortic regurgitation.
- Reactive arthritis related to infections. Septic arthritis
- Septic arhritis
This is a more traditional infection of the joint space, hematogenous spread of bacteria (often Staphylococcus aureus, and before immunization, H.influenza) into the synovial fluid of the joint. This will cause a local infection with an erythematous, warm and acutelytender joint with limited range in movement. The younger the child the more likely this is to present with generalized illness.
Investigation includes CBC and acute-phase protein investigation, an Ultrasound may also be useful to assess effusion, while X-rays can be used to exclude trauma if the history suggests it. Treatment is the washing out of the joint with surgical drainage and a prolonged course of antibiotics required (possibly IV). Joints such as the hip are more dangerous and the patient should ensure they they work on their mobilization of the joint else a long term deformity may occur.
The diff dx includes lyme disease, osteomyelitis, suppurative bursitis, fasciitis, myositis, cellulitis and soft tissue abscesses.
Infectious arthritis secondary to bite wounds
Infection due to human, dog, or cat bites (see Human and Mammal Bites) usually develops within 48 hours.
Rat bites cause systemic symptoms such as fever, rash, and joint pain or true arthritis with regional adenopathy within about 2 to 10 days.
Viral infectious arthritis
Viral infectious arthritis sometimes causes symptoms similar to acute nongonococcal bacterial arthritis and is more likely to be polyarticular than bacterial arthritis.
- Juvenile idiopathic arthritis
- Generall
Juvenile idiopathic arthritis is a group of rheumatic diseases that begins by age 16. Arthritis, fever, rash, adenopathy, splenomegaly, and iridocyclitis are typical of some forms. Diagnosis is clinical. Treatment involves intra-articular corticosteroids and disease-modifying antirheumatic drugs.
- Juvenile idiopathic arthritis
- Classifications
Juvenile idiopathic arthritis is not a single disease; the term applies to a number of chronic, noninfectious arthritides that occur in children and share certain features. The current classification system, from the International League of Associations for Rheumatology, defines categories of disease based on clinical and laboratory findings. Some of the categories are subdivided into different forms. Categories include the following:
- Oligoarticular JIA (persistent or extended)
- Polyarticular JIA (rheumatoid factor [RF] negative or positive)
- Enthesitis-related arthritis
- Psoriatic JIA
- Undifferentiated JIA
- Systemic JIA
- Juvenile idiopathic arthritis
- Etiology
Juvenile idiopathic arthritis (JIA) is uncommon. The cause of JIA is unknown, but there seems to be a genetic predisposition as well as autoimmune and autoinflammatory pathophysiology. The vast majority of children with JIA have a disease that is distinct from adult rheumatoid arthritis (RA), but in 3 to 5% of children with JIA, the disease is analogous to adult RA
- Juvenile idiopathic arthritis
- Oligoarticular JIA (persistent or extended)
- Polyarticular JIA (rheumatoid factor [RF] negative or positive)
- Enthesitis-related arthritis
- Oligoarticular JIA is the most common form and typically affects young girls. It is characterized by involvement of ≤ 4 joints during the first 6 months of disease. Oligoarticular JIA is further divided into 2 types: persistent (always ≤ 4 joints involved) and extended (≥ 5 joints involved after the first 6 months of disease).
- Polyarticular JIA is the second most common form. It affects ≥ 5 joints at onset and is divided into 2 types: RF negative and RF positive. Typically, young girls are RF negative and have a better prognosis. The RF-positive type typically occurs in adolescent girls and is analogous to adult rheumatoid arthritis. In both types, arthritis can be symmetric and frequently involves the small joints.
- Enthesitis-related arthritis involves arthritis and enthesitis (painful inflammation at the insertion of tendons and ligaments). It is more common among older boys, and these patients may subsequently develop arthritis of their axial skeleton (sacroiliac and lumbar spine). Enthesitis-related arthritis tends to be in the lower extremities and asymmetric. The human leukocyte antigen–B27 (HLA-B27) allele is more common in this form of JIA.
- Juvenile idiopathic arthritis
- Psoriatic JIA
- Undifferentiated JIA
- Systemic JIA
- Psoriatic JIA has a bimodal age distribution. One peak occurs in young girls, and the other peak occurs in older males and females (who are equally affected). It is associated with psoriasis, dactylitis (swollen digits), nail pits, or a family history of psoriasis in a 1st-degree relative. Arthritis is frequently oligoarticular.
- Undifferentiated JIA is diagnosed when patients do not meet criteria for any one category or meet criteria for more than one.
- Systemic JIA (Still disease) involves fever and systemic manifestations.
- Juvenile idiopathic arthritis
- clinical manifestations
- Diagnosis
- Children typically have joint stiffness, swelling, effusion, pain, and tenderness, but some children have no pain. Joint manifestations may be symmetric or asymmetric, and involve large and/or small joints. Enthesitis typically causes tenderness of the iliac crest and spine, greater trochanter of the femur, patella, tibial tuberosity, Achilles insertion, or plantar fascia insertions.
- Skin abnormalities are present mainly in psoriatic JIA, in which psoriatic skin lesions, dactylitis, and/or nail pits may be present, and in systemic JIA, in which a typical transient rash often appears with fever. Rash in systemic JIA may be diffuse and migratory, with urticarial or macular lesions with central clearing.
- Systemic abnormalities in systemic JIA include high fever, rash, splenomegaly, generalized adenopathy (especially of the axillary nodes), serositis with pericarditis or pleuritis, and interstitial lung disease. These symptoms may precede the development of arthritis. Fever occurs daily (quotidian) and is often highest in the afternoon or evening and may recur for weeks. In 7 to 10% of patients, systemic JIA may be complicated by macrophage activation syndrome, a life-threatening cytokine storm syndrome.
- Juvenile idiopathic arthritis
- Diagnosis
Clinical evaluation
-Rheumatoid factor (RF), antinuclear antibodies (ANA), anticyclic citrullinated peptide (anti-CCP) antibodies, and HLA-B27 tests
Juvenile idiopathic arthritis should be suspected in children with symptoms of arthritis, signs of iridocyclitis, generalized adenopathy, splenomegaly, or unexplained rash or prolonged fever, especially if quotidian. Diagnosis of JIA is primarily clinical. It is made when a chronic noninfectious arthritis lasting > 6 weeks has no other known cause.
- Patients with JIA should be tested for RF, anti-CCP antibodies, ANA, and HLA-B27 because these tests may be helpful in distinguishing between forms. The test for ANA should be done by immunofluorescence because other methods may result in false-negative results.
- In systemic JIA, RF and ANA are usually absent. In oligoarticular JIA, ANA are present in up to 75% of patients and RF is usually absent. In polyarticular JIA, RF usually is negative, but in some patients, mostly adolescent girls, it can be positive. HLA-B27 is present more commonly in enthesitis-related arthritis. In systemic JIA, laboratory abnormalities suggestive of systemic inflammation, such as elevated erythrocyte sedimentation rate (ESR), ferritin, and C-reactive protein, along with leukocytosis, anemia, and thrombocytosis are almost always present at diagnosis.
- To diagnose iridocyclitis, a slit-lamp examination should be done even in the absence of ocular symptoms. A recently diagnosed patient with oligoarticular or polyarticular JIA should have an eye examination every 3 months if ANA test results are positive and every 6 months if ANA test results are negative.
- Juvenile idiopathic arthritis
- Treatment
- Slow disease progression with methotrexate and/or biologic drugs (eg, etanercept, anakinra, canakinumab, tocilizumab) and treat symptoms with intra-articular corticosteroid injections and/or NSAIDs.
- Treat iridocyclitis with ophthalmic corticosteroid drops and mydriatics, or systemic therapy if refractory.
- Methotrexate is useful for oligoarticular, psoriatic, and polyarticular forms of JIA. Adverse effects are monitored as in adults. Bone marrow depression and hepatic toxicity are monitored with complete blood count, aspartate aminotransferase (AST), alanine aminotransferase (ALT), and albumin. Occasionally, sulfasalazine is used, especially in cases of suspected spondyloarthritis.
- TNF inhibitors are used if methotrexate is not effective. Etanercept is used most commonly at doses of 0.8 mg/kg subcutaneously (up to 50 mg) once a week. Adalimumab and infliximab are other TNF inhibitors that have been shown to be effective. The IL-1 inhibitors anakinra or canakinumab are particularly effective for systemic JIA. Tocilizumab, an IL-6 receptor antagonist, is also indicated for the treatment of systemic JIA.
Macrophage Activation Syndrome
-Macrophage activation syndrome is hemophagocytic lymphohistiocytosis associated with juvenile idiopathic arthritis (JIA; particularly systemic JIA) and with several other rheumatic diseases (particularly adult-onset Still disease).
Macrophage activation syndrome is a severe, overwhelming, and life-threatening cytokine storm syndrome caused by an uncontrolled macrophage and T lymphocyte expansion. It occurs in 10% of children with systemic JIA.
-Symptoms and Signs of Macrophage Activation Syndrome
Features macrophage activation syndrome include high, nonremitting fever, hepatosplenomegaly, generalized lymphadenopathy, rash, hemorrhagic manifestations, central nervous system dysfunction (including seizures and coma), and shock.
-Diagnosis of Macrophage Activation Syndrome
Features that help distinguish macrophage activation syndrome from systemic JIA include the constant fever (unlike the intermittent daily fevers of JIA), constant rash (unlike the transient rash of systemic JIA), hemorrhagic manifestations, seizures, coma, and shock. Despite marked systemic inflammation, the erythrocyte sedimentation rate (ESR) is paradoxically depressed because of low fibrinogen levels.
-Treatment of Macrophage Activation Syndrome
No consensus exists regarding specific treatment.
Macrophage activation syndrome may respond well to successful treatment of the underlying rheumatic disease. Specific treatment of this syndrome in systemic JIA usually includes high-dose corticosteroids and may include other drugs (eg, cyclosporine, interleukin [IL]-1 inhibitors, cyclophosphamide) and stem cell transplantation.
- Systemic lupus erythematosus
- General
-Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. Common manifestations may include arthralgias and arthritis, Raynaud syndrome, malar and other rashes, pleuritis or pericarditis, renal or central nervous system involvement, and hematologic cytopenias. Diagnosis requires clinical and serologic criteria. Treatment of severe, ongoing, active disease requires corticosteroids and immunosuppressants.
