Pediatric rheumatic disease Flashcards
What are the typical features of adult Still’s disease?
Still’s disease usually involves the knees, ankles, wrists more than the small joints and is often associated with pharyngitis, and lymphadenopathy. Biopsy shows B cell hyperplasia. Hemophagocytic syndrome is a rare complication and accounts for most deaths. Interstitial lung disease and myocarditis can happen. White cell counts over 15,000 and elevated ferritin levels are common. Biopsy of the skin rash shows complement deposition.
What are the diagnostic criteria for adult Still’s disease.
The Yamaguchi criteria for adult Still’s disease include four major criteria: 1. fever at least 39°C2. arthralgia or arthritis lasting two weeks or longer3. nonpruritic skin rash which is maculopapular and salmon colored found on the trunk and extremities during febrile episodes4. leukocytosis exceeding 10,000 with at least 80% granulocytes. Minor criteria include sore throat, lymphadenopathy, hepatomegaly or splenomegaly, elevated AST or ALT, and negative testing for ANA and rheumatoid factor. Criteria are satisfied if at least 5 items are present including at least 2 major criteria
What are the diagnostic criteria for Behcet’s disease?
Behcet’s disease is characterized by the presence of recurrent aphthous ulcers with at least 3 episodes in any 12 month period. There must be at least 2 additional features such as: recurrent genital ulceration, eye lesions, skin lesions, or positive pathergy test. Anterior or posterior uveitis on slit lamp examination or retinal vasculitis documented by an ophthalmologist, erythema nodosum or papulopustular skin lesions or pseudo-folliculitis with characteristic achneiform nodules observed by a physician. Other features commonly explained by Behcet’s disease include arterial aneurysms in the heart, kidney, or pulmonary vasculature, oligo or polyarticular non deforming arthritis, renal amyloidosis or glomerulonephritis, pericarditis or myocarditis. A positive pathergy test is defined by a papule 2 mm or more mm in size developing within 48 hours after the insertion of a 20gauge needle 5 mm into the forearm skin. Aseptic meningoencephalitis, cerebral vasculitis, recurrent phlebitis, arteritis, synovitis, or focal bowel ulceration might substitute for pathergy in European or North American populations.
What commonly causes bilateral knee pain in an adolescent with normal x-rays?
Billateral anterior knee pain is a descriptive diagnosis for a condition formally called chondromalacia patella. The descriptive term is more honest as chondromalacia is difficult to demonstrate as a specific cause of knee pain and is often asymptomatic. Bilateral anterior knee pain seems to occur in late adolescence during vigorous physical activity and then subsides over years. It is not a precursor to osteoarthritis or rheumatoid arthritis. Mechanical irritation in the soft tissues guiding the quadriceps/patellar tendon from overuse and/or poor biomechanical stress tolerance is the probable cause.
With systemic diseases can cause muscle enzyme elevation?
Systemic disorders such as inflammatory myopathy (polymyositis/dermatomyositis, systemic lupus erythematosus, and mixed connective tissue disease with antibodies to RNP), infectious myopathy, drug induced myopathies (alcohol, lipid lowering drugs, cocaine, antimalarial drugs, antipsychotic drugs, colchicine, antiretroviral drugs, ipecac, Interferon alpha and penicillamine), dystrophic myopathies, neuroleptic malignant syndrome, metabolic myopathies, hyperthermia, motor neuron disease, endocrine myopathy, and periodic paralysis should be considered. Presymptomatic myopathies as in the early stages of polymyositis, distal myopathy, mitochondrial myopathies, inclusion body myopathy, muscular dystrophy, myotonic dystrophy, and predisposition to malignant hyperthermia.
What is the PFAPA syndrome?
PFAPA syndrome refers to a pediatric condition usually presenting before age 5, consisting of recurrent pharyngitis, cervical lymphadenopathy, and aphthous stomatitis without correlation to ethnicity, geographical distribution, or gender or genetic defect.
What is Erdheim-Chester disease?
Erdheim-Chester disease, a.k.a. polyostotic sclerosing histiocytosis is characterized by lipid laden macrophages, multinucleate giant cells, and inflammations in the bone marrow with generalized sclerosis of long bones. Biopsy showing CD68+ CD1a- cells is typical.
What is the usual initial treatment for polymyositis, and dermatomyositis?
Initial treatment of polymyositis, dermatomyositis, and inclusion body myositis are similar. A course of prednisone at 1 mg per kilogram (Max 80 mg) daily for six weeks followed by reduction (10 mg per week until 40 mg daily, then 5 milligrams per week until 20 mg daily, 2.5 mg per week until 10 mg daily, then 1 mg every two weeks until 5 mg daily). Responders may be off treatment by one year. Azathioprine at 2.5 mg per kilogram or methotrexate at 25 mg per week is usually used along with low-dose prednisone (5 to 15 mg daily).
What are the symptoms of celiac disease?
Frequent symptoms include chronic diarrhea and weight loss, double distention, iron deficiency, aphthous stomatitis, recurrent abdominal pain, short stature, high aminotransferase levels, chronic fatigue, and reduced bone density. Unusual symptoms include dermatitis herpetiformis, gluten ataxia. Untreated disease may develop splenic dysfunction, osteoporosis, neurologic disorders, infertility or recurrent abortion, ulcerative jejunoileitis, T-cell lymphoma and adenocarcinoma.
What are some common amyloid types?
Aβ, Beta amyloid, forms in Alzheimer’s disease, AA from SAA in Rheumatoid Arthritis, ACal-calcitonin in medullary cell carcinoma, Aβ2M Beta-2 microglobulin in dialysis related amyloidosis, and alpha-synuclein in Parkinson’s disease.
What common diseases cause arthritis in children?
The following conditions are considered likely: JIA, parvovirus B19, Lyme disease, SLE, then less likely causes include rheumatic fever, chronic infectious arthritis, subacute bacterial endocarditis, tuberculous arthritis, fungal arthritis, and Whipple’s disease.
Who is at risk to develop fluoroquinolone induced musculoskeletal toxicity?
Damage increases with higher loads across cartilage. Risk is higher if elderly, and concurrent use of gluceocorticoids. Achilles rupture occus in 17/100,00.Prescription event monitoring gives an incidence of 2.4/10,000 for tendinitis, and 1.2/10,000 or tendon rupture. Relative risk was 3.2 if greater than 60 and 20 if over 80 years of age, increasing to 6.2 in patients over 60 taking glucocorticoids. Incidence is affected by dose but not duration of treatment. Fluoroquinolone treatment results in Achilles tendon rupture in 1/6000 patients, 1/1638 if over 60, and 1/979 if taking glucocorticoids.
What laboratory tests help determine the course of JRA?
In 227 Italian patients with JRA disability was predicted by articular severity score (OR 5.7) and the best was positive ANA (OR 0.3). Early hand involvement-OR 8.7, HLA-DR 5 was the strongest predictor for pain-OR 3.3. 1997
