Once you have a suspected area or limb, concentrate on that - check bones in between joints esp metaphyses for pain (marrow infiltration).
MRC/Oxford scale of strength:
Start assessing at 3. Score only applies if strength maintained throughout patient's full range of movement, and without trick movements. Note high interobserver variability at 4 and 5. Dynamometer a bit more objective for grip/pince at that end of the range.
Childhood Myositis Assessment Scale (CMAS) is a useful way for looking at function, esp in younger kids, with or without myositis: lift head from bed, sustained straight leg raise (SLR), roll from supine to prone, sit up, straight arm raise, stand to sit, pick up object from floor etc.
Beighton scales for hypermobility - scores points (out of 9) for:
Bone scan (DXA, uses 2 different wavelengths of X-ray) gives bone mass/area (as well as lean and fat mass), so underestimates in small children cf mass/volume. Need to correct eg by bone area. Ultrasound assessment under evaluation (measures speed of sound along fixed length of radius or tibia).
Calcium suppl of limited benefit (?assoc with Genotype), vigorous exercise similarly in healthy kids. Vit D explored by Drs Goel/Angus Ford.
Difficult to define osteopenia on Xray. Often assoc with growth retardation, delayed puberty (all steroid assoc). Monitor ht, sitting ht, puberty. DXA if available, Vit D. Use alternate day steroids. NB methotrexate/cyclosporin still have bone effects. Consider sex steroids/GH.
Exclude the nasties:
Beware back pain if <4yrs, night time, leading to postural shift or functional disability
Plus some odd ones:
The most pressing to exclude. Presentation is acute, child is toxic, with hot/swollen/tender joint held in position of least discomfort. Osteomyelitis more subacute, with tender bone rather than joint, loss of function, no fever. Tend to have less effusion but more focal tenderness. Beware: parents often recall minor injury which may be the reason why bugs got in, but may also put you off track. Beware (2): more than 1 joint may be involved! Chronic symptoms, early morning stiffness suggest JIA but may be TB. Given that there is a high incidence of culture negative disease, surgical specimens should be considered for culture and histology.
Investigate the sick child with:
Main organisms are: Hib, Staph, Strep but also gram negatives in neonates including neisseria, salmonella. Kingella newly recognized. But unknown in up to half, wide geographical variation, depends on whether you use PCR vs culture. Beware penetraing injuries with risk of contamination (producing bizarre organisms). Treat with Cefuroxime/Ampicillin and Fluclox, short courses even oral effective if uncomplicated. Fucidin use is falling out of favour... Ceftriaxone not always great against staph, but you could switch after a couple of days of cefuroxime. Consider serial aspiration and prolonged (oral) courses if complicated. Hips should always be drained, otherwise consider surgery if symptoms persist for more than 5 days. Potential complications are septicaemia, bone/cartilage death, deformity, pain...
Or Irritable hip. Young child, may be history of URTI, goes off their feet. May well be spasm, reduced range of movement, small effusion, but afebrile and looks generally well. XRs are of little use without a history of trauma, unless prolonged symptoms that might indicate occult fractures or Perthes disease. Early follow-up is probably the most important way to avoid missing a septic arthritis.
Treat with bed rest... child will eventually get restless...
=osteochondritis, ?cause. Boys mostly, youngish eg 3 to 11 yr. There may be a family history, it's more common in white, esp Irish, socially deprived populations! Presents with limp, but not much pain, reduced internal rotation. XR changes are characteristic (shows crescentic subchondral lucency, particularly in the anteromedial aspect, best seen on lateral; late changes are flattening, sclerosis and fragmentation); but MRI and bone scan are more sensitive. Treatment ranges from not much to bed rest/traction to osteotomy, but most will have normal function in adult life, even if the XR is never normal.
Also more common in boys, but affects older boys (over 10 yr) and is associated with obesity and delayed sexual development. Seems to be a hormonal imbalance. Affected girls tend to be younger, matching their growth spurt. Tends to be more painful than Perthes although findings ie short leg, held in external rotation, reduced internal rotation are same. Best XR is Frog legged lateral. Treat by pin or osteotomy, and remember to follow up other hip.
Seven subtypes - only diagnose when symptoms for at least 3 months:
Clinically, history of pain, swelling, stiffness. Pain is usually not severe, and often avoided completely by adapting movement; can occur at night, and occurs in the joint line. Degree of pain does NOT predict severity of synovitis. Swelling may be due to effusion or bony overgrowth. Stiffness not so severe as to cause gelling ie sitting still leads to freezing (cf myasthenia gravis, hypermobility). Bony overgrowth, discrepant leg length (longer with inflammation! Leads to postural scoliosis), wasting show chronicity.
Differentials:
Investigations:
Can be systemically very unwell and potentially life threatening complications may occur early in the disease course (eg pericarditis, macrophage activation syndrome or HLH, sepsis). Start high dose corticosteroids after careful exclusion of other diagnoses, especially infection, Kawasaki disease, and malignancy - difficult when arthritis is absent.
Systemic features may predate the arthritis by several weeks and occasionally longer. Typically involves small joints of the hands and wrists, ankles, hips, knees, and cervical spine - about 30% ultimately develop severe polyarthritis.
There are no pathognomic tests or agreed diagnostic criteria for SOJIA! Exclude other conditions, look for classic features:
Treat with IV methylprednisolone pulses (30mg/kg over 4 hours, max 1g, once daily for 3 days) and ibuprofen (seems better than piroxicam for SOJIA!). Oral prednisolone may then be used while methotrexate is introduced.
NSAIDs and intra-articular steroids work quickly. Ibuprofen can be given at high dose (10mg/kg qds), else Diclofenac 3-5mg/kg in 3-4 divided doses, max 150mg. Piroxicam is once daily, which is convenient but it probably has more GI/cutaneous side effects. No longer considered appropriate for acute pain. Routine NSAIDs are probably pointless; if you need regular anti-inflammatories, you should probably be on a disease modifying agent eg methotrexate. Joint injections are given under general anaesthetic in young children or with entonox in older children. Lederspan (triamcinolone) 1mg/kg max 40mg used for big joint, 0.5mg/kg for wrist, TMJ. Knuckles will only take 0.1-0.2ml before they start to leak (which leads to subcut atrophy). Injecting multiple (eg >6) sites can result in Cushings for 3-6/12. Better to pulse methylpred? (Kennilog is another formualtion, but seems to give more Cushings). Most patients tolerate injections well and have no loss of function immediately after; physio is usually started after 24hr. How often? Balance of steroid effects and uncontrolled joint disease...
Methotrexate Side effects: GI (nause, ulceration, diarrhoea), hepatotoxicity (reversible elevations of serum liver enzymes eg 3x upper limit normal common), Pulmonary (oedema, pleuritic pain, pulmonary fibrosis, interstitial pneumonitis), mood changes, Renal (haematuria, dysuria, renal failure) - plus usual chemo stuff ie bone marrow suppression.
Methotrexate is the disease modifying drug of choice - early use helps to reduce joint damage and minimise the exposure to, and side effects of, corticosteroids. Well tolerated in most children but often causes nausea the day after administration (so usually given on Friday to avoid affecting school). Avoid alcohol, as potentiates risk of cirrhosis. Takes 6-8 weeks to become effective so cover interval with intravenous methylprednisolone. The theoretical risk of malignancy and infertility has not so far been borne out in long term outcome studies. Folic acid improves tolerability but not clear what regimen to use - BNF suggests 5mg once weekly or 1mg daily, theoretically it should not be given within 24 hours of the MTX [so once weekly sounds easier]. Methotrexate is given once a week at 10-25mg/m2 - can be oral but subcut route improves bioavailability at doses beyond 10mg/m2. Metojet has better shelf life (10 months). Regular blood tests to monitor inflammatory markers and side effects eg monthly for 6 months then 3 monthly thereafter. Not great for axial disease ie HLA B27.
Steroids are useful for treating acute flares. Methylprednisolone can be given once daily for 3/7 to control severe exacerbations, then once weekly thereafter (30mg/kg, max 1g). Don't work well for axial disease though ie HLA B27 (although may be good for peripheral joints) - TNF blockade (ie etanercept or infliximab) effective.
Patients who are refractory to high dose parenteral methotrexate are considered for novel immunosuppressive agents such as Etanercept/infliximab (TNF antibody), autologous stem cell transplantation, or very high dose immunosuppression.
Calcium and vitamin D supplements are often given for bone health.
Patients on immunosuppressants should avoid live vaccines and beware of infection. If unwell enough to need antibiotics they should probably stop treatment temporarily. Varicella is a particular concern - if contact with chickenpox and non-immune, consider VZIG for prophylaxis, and IV aciclovir for treatment. See Greenbook.
Not clear when to wean... Many patients do well for a year or so before their condition begins to worsen, swapping to another agent often works, and swapping back is also a useful option.
JIA is a not a benign disease and outcome is variable. At least a third of patients have ongoing active disease into adulthood and many have sequelae eg:
Bisphosphonates seem to be effective for increasing bone mass in JIA. Flu-like symptoms with first IV dose can be treated with paracetamol and tend not to recur.
Occurs in about 10% of patients with non-oligoarthritis, and 30% of ANA positive oligo so pretty common. Almost always asymptomatic, but can lead to blindness due to cataract, glaucoma, synechiae etc. So they all need screening, pref by someone with paediatric experience, and within 6 weeks of diagnosis - most avoidable morbidity due to uveitis developing before or at the time of diagnosis.
Regular screening only dropped when child able to report subtle changes in vision themselves - but still advised to monitor monocular visual acuity. Or when joint disease has been quiescent for at least 7yrs! Symptoms would be visual loss, photophobia, corneal clouding; possibly manifest as unusual rubbing, blinking, new onset squint.
Treat with topical/systemic steroids, MTX, MMF (although not great for joint disease), infliximab (seems better than etanercept).
Should include nurse specialist, physiotherapist, occupational therapist, paediatric rheumatologist, ophthalmologist, clinical psychologist, social worker, dentist, orthodontist, and orthopaedic surgeon.
Stopping methotrexate: 50% flare after 1yr remission, 50% after 2yr - 3yrs? Presumably some will be chronic, and some will not.
Thumbs may be spared. Triphasic response (white/blue/red). ?Nifedipine, ?modified release. Rarely CT disease follows: SLE, systemic sclerosis esp CREST.
Usually anaemia, thrombocytopenia. Lots of autoantibodies (see above), may be low complement. Anti dsDNA and antiphospholipid are actually pathogenic, not just markers. Antiphospholipid antibodies are common in SLE, and there is a significant relationship between the presence of a lupus anticoagulant, but not anticardiolipin antibodies, and thrombotic events.
Neonatal lupus erythematosus presents with cutaneous lesions, complete heart block, hepatic disease, and thrombocytopenia. Cranial US and CT in the first 2 months often abnormal even without neuro symptoms eg attenuated white matter, basal ganglia calcifications (prob self resolving). IgG anti-Ro and/or La antibodies cross the placenta and cause the clinical manifestations. Mum may not be known to have lupus - but she is likely to develop disease in time. Although antibody will be cleared over a few months, heart block may persist, with 63% eventually requiring pacemakers. Babies with NLE are also at risk of developing other autoimmune diseases during childhood. Current Opinion in Rheumatology. 13(5):415-21, 2001
Minocycline and zafirlukast have been associated with drug induced lupus.
Porphyria is the most important differential, esp where disease is drug-induced or photosensitive. Acute hepatic porphyrias present with:
Porphyria attacks require intervention with intravenous glucose, heme preparations and the discontinuation of dangerous porphyrinogenic drugs including antiseizure drugs.
Prednisolone and hydroxychloroquine are starting treatments. Methotrexate doesn’t seem to be work well in paed SLE although it does in adults. Cyclophosphamide is used in monthly pulses for more severe disease (should not be used in those with renal impairment), most children tolerate short-term therapy but toxicity profile ranges from mild to moderate hair loss, nausea, immune suppression, and a risk of developing permanent ovarian failure. If followed by maintenance with MMF or azathioprine, adults with proliferative nephritis do better than with cyclophos alone. Rituximab (anti-CD20, knocks out B cells) works for resistant cases.
V complex!
Although vaccines can reduce the risk of certain types of infection, it is possible that immunizations with live viruses may result in disease flares
Advice and instruction on photoprotective measures. E45 gives noticeable colour, ROC is better screen. Neon lights can produce photosensitivity.
Later problems are bone health eg avascular necrosis (due to chronic steroid use) and hypertension (chronic steroid use plus nephritis).
In adults with any sort of chronic inflammatory disease, cardiovascular risk is increased. In women with SLE 35 to 44 years of age, the incidence of myocardial infarction is 50 times greater than for women without the disease. The increased incidence of cardiovascular disease is not explained by traditional risk factors alone: lipid metabolism is certainly disturbed, homocysteine levels are elevated but all complicated by inflammation, drug effects, antiphospholipid syndrome, renal involvement. The efficacy of antihyperlipidemic agents and folic acid therapy (for reducing homocysteine levels) in children has not been proven.
Scleroderma is a spectrum:
Look for "botox like" forehead, bird like face, sclerotic skin, nailfold changes, telangiectasia, calcinosis. May also get myositis, dry synovitis (ie little inflammation), GI probs, hypertension & proteinuria, arrhythmias, pulmonary fibrosis. Most have some auto-antibodies esp Centromere (Crest), Extractable nuclear antibodies (ENA) esp anti-Scl-70 (Sclerosis). Variable erratic progression.
Screen with ECG and lung function.
Differential is polymyositis (same thing without the skin involvement) and mixed CT disease (ie features of lupus etc). Features:
Rash can progress to panniculitis with lipoatrophy. CK may be normal! But look for raised AST/ALT, LDH, Aldolase (ie other muscle enzymes), ESR. These
markers are no use for monitoring disease progression. Nailfold
capillaroscopy can be diagnostic. Do MRI of upper thigh or affected
muscle, usually obviates need for biopsy of affected muscle (more
sensitive than US, EMG). Autoabs present in 12% - if pos, clinical
syndrome overlaps more with scleroderma. Monitor CMAS (childhood myositis assessment score, out of 53), global extramusc disease score, serial MRI.
40% have acute, unicyclic pattern (ie one off, burns out).
Methotrexate and pulsed methypred, consider ciclosporin (hypertension, reduced GFR, hirsutism), cyclophosphamide, IVIG (block Ig receptors, block autoabs, modify cytokines),
Mycofenolate mofotil (MMF), etanercept/infliximab (anti TNF) for most
severe - all effective. Consider bisphosphonates for calcinosis
(and steroid related osteoporosis). For rash - topical
steroids/hydoxychloroquine/tacrolimus, sunblock (E45 works but looks horrible, Sunsense or Roc 25 available on prescription). Skin disease often
relatively resistant cf muscle disease, poor control is a reason to escalate treatment. Plasmapheresis for life-threatening disease.
Look out for pulmonary fibrosis, cardiomyopathy, arrhythmias, dysphagia. Panniculitis may represent increased inflammation, but can also be infective (eg staph).
Back ache - beware young children, night pain, loss of function. Posture - is the head forward, with kyphosis or lordosis? May represent tight hamstrings or weak abdominal muscles. Is there kyphosis without lordosis, suggestive of congenital kyphosis or Scheuermann's disease? Schobers test for lumbar spine movement (draw points 10cm above, 5cm below line of SIJs - normally increases with flexion to 21+cm). Extension lost early in inflammatory disease.
Treat biomechanical pain with physio - posture, strengthening eg pelvis/abdomen/back, flexibility.
Distinguish from positional plagiocephaly - in the latter, ear on flat side comes forward cf lambdoid stenosis - bone hypoplastic, so ear behind. Scaphocepahly does not lead to RICP, but deformity can be severe (frontal & occipital bossing - peanut appearance) so cosmetic reasons for surgery @ 6/12.
Look for facial scoliosis - unilateral coronal fusion causes curved midline structures, so eyebrows different heights. Unilat frontal bossing looks similar to positional plagio. Sxr shows "harlequin eye" - sup lat corner distorted outwards, +/- copper beating of affected side (sutures difficult - can be asymmetrical in plagio). Trigonocephaly (premature metopic suture fusion, in middle of forehead) usually fixed @ birth; rarely RICP. Dolicho is same as brachy!
RICP usually periodic. Presents with nighttime restlessness - goes down Ok, but wakes screaming. Headbanging is a clue, esp if small OFC.
See Vasculitis under Immunology.
Formerly known as congenital dislocation of hip (CDH). Associated with family history, breech lie.
Most detected in neonatal period: asymmetrical groin creases, Ortolani-Barlow manoeuvre will reveal instability. But beyond 6/52 of age, instability less obvious, and walking may not be delayed. May present as limp, or odd gait. Examine leg length (flex hip to 90deg, compare height of knee). Abduction will be restricted.
Also known as wry neck. Describes a twisted head position. Not a diagnosis. Different aetiologies in infants compared with older children/adults.
Sternomastoid tumour is a terrible name for a thickened area of the sternomastoid muscle causing shortening and hence torticollis. Originally thought to be due to birth trauma but this is difficult to prove and it may represent an antenatal or postnatal event. [suggesting birth trauma as a cause of anything is always dangerous ground). Congenital muscular torticollis is a better name for the same thing. Most common cause of torticollis in infants, can be associated with plagiocephaly and facial asymmetry. Diagnose by ultrasound. Improves with physiotherapy in most cases, especially if instituted within the first year of life. Botox is effective but obviously it depends how long standing (hence reversible) the torticollis is. If it fails to improve, a surgical release of the muscle may improve the range of motion, but not necessarily the plagiocephaly and facial asymmetry.
Dudkiewicz I et al. Journal of pediatric orthopedics. 2005 Nov-Dec; 25(6):812-4. PMID 16294141. Do TT. Current opinion in pediatrics 2006;18(1):26-9. PMID 16470158
Congenital spinal anomalies causing torticollis are rare, and 1 study suggested that plain films in this setting produces more false than true positives. In any case, CT is probably better. Pediatrics 2006;118(6):e1779-84. PMID 17116697
Benign paroxysmal torticollis typically begins in the first months of life. Episodes may last hours or days, and sometimes can be associated with other symptoms:
Tends to disappear spontaneously after some months, and usually by the age of 5 years. Diagnosis is on the clinical pattern and course, plus by exclusion of other pathologies.[Revista de neurologia. 2006 Sep 16-30; 43(6):335-40. PMID 16981163
Rarely a manifestation of atlantoaxial disturbance secondary to trauma or oropharyngeal inflammation (=Grisel syndrome eg tonsillitis). Consider also:
More usually a not uncommon spontaneous presentation eg on waking up. ?vertebral facet joint pinching its capsule. Gentle exercise and manipulation of the neck (applying a little upward traction) usually helps.
Herman MJ. Instructional course lectures 2006;55:647-53. PMID 16958498
Vitamin D deficiency, usually due to poor maternal supply in pregnancy, then reduced sun exposure and diet (esp milk). Else fat malabsorption. Classically bowlegs, body pains, pigeon chest, rachitic rosary, skull deformity, kyphoscoliosis, poor dentition. XR wrist is most useful, shows cupped, frayed metaphyses. Check vitamin D levels - low calcium and high alkaline phosphatase may suggest. Once treatment with Vitamin D starts, calcification makes these abnormalities more apparent.
A syndrome of chronic pain, with hypersensitivity and vasomotor instability (colour/temp/sweating), due to disuse eg post fracture. Hence usually isolated. Treatment is mobilisation else sympathectomy.
Beighton scale as above. Can be seen in Ehlers-Danlos, Marfan and Stickler (myopia, retinal detachment) syndromes. Transient swelling. Physio doesn't help much! Chronic pain team may need to be involved. Often central nervous system comorbidity seen eg dyspraxia, Aspergers - so central origin for hypermobility?
Ehlers-Danlos type 3 is benign joint hypermobility. Pain responds poorly to conventional analgesics. Type 4 is the rare but lethal autosomal dominat form where risk of arterial, bowel and uterine rupture, mostly between ages 20-40. Sheffield does genetic testing. History of hernia repair?
Chronic inflammatory condition found classically in people from the Silk Road (Central Asia to the Mediterranean via the Middle East and Turkey), but does not have any specific genetic or tissue type markers and can be found in all races. Not your typical autoimmune disease either.
Cardinal features are:
Common problems:
Treatments incl infliximab (esp for uveitis), etanercept (esp for skin), azathioprine, colchicine, thalidomide.
See also Septic arthritis, above. XR in infection shows mixture of fast (cortical breach, lysis) and slow (sclerosis, periosteal reaction) changes. Abscess forming sinus into soft tissue is indicative of infection, the other appearances are non-specific. Sequestrum is isolated bit of dead bone. Onion skin appearance suggests chronic infection but is also seen in normal infants, malignancy and in retinoid therapy. But these appearances take 1-2 weeks plus to develop. USS may show sub-periosteal oedema early on, but MRI standard for defining collections and guiding surgery: 92-97% sens cf 64-71% for bone scan (latter is also operator dependent).
In neonates, approx 50% have no systemic features, present with pseudoparalysis alone. Pelvic often present with abdo/lumbar pain.
Staph aureus is a common cause, plus:
But unknown in up to half, geographical variation, PCR vs culture...
Traditionally 6 weeks of IV treatment, but not very good evidence! Peltola treated Staph for a total of 3 weeks guided by CRP (falls rapidly), switching from IV to oral after 3-4 days, using high doses of cephradine or clindamycin. They had no cases of relapse at 1 year (n=50). Peds 1997 PMID 9190554
More difficult if no bug isolated. Failure rates of 19% have been found where duration was under 3 weeks, but not sure if this was guided by CRP/ESR (ESR takes longer to fall, as usual). A Canadian study group found no difference between short (<=7 days) and long courses of IV for Staph infection.
Until better evidence available, IV treatment should be initiated, then switched to high dose oral after 3-4 days as long as CRP has normalised. Total course should be at least 3 weeks. Curr Op Inf Dis 2008 PMID 18448970
CA-MRSA most common in US, clinically the same disease but if Panton Valentine Leucocidin positive then more febrile, multiple sites, chronic. Vancomycin is used; efficacy of Septrin for bone not estalished. Clindamycin good if resistance rates low - added benefit in MSSA? Added benefit of rifampicin?
No RCTs for duration - ?ESR<30 (usually 3-4 weeks). If bug unknown, then difficult. Kingella, incidentally, is resistant to vanc and clinda! But can be treated with Ceftriaxone.
Surgery is an essential adjunct to antibiotics for neonates, chronic, collections, hip/shoulder, puncture associated. Interventional radiologist?
Often complicated by deep vein thrombosis (DVT) esp staph, esp PVL. Once endovascular infection has occurred, disseminated infection and persistently positive blood cultures may result. Do Echo if persistent bacteraemia with sensitive bug in case of endocardial infection. Vancomycin theoretically more appropriate for endovascular infection than clindamycin: Rx as IBE. Linezolid fairly effective in compassionate use series. Daptomycin is most rapidly bacteriocidal in vitro, reported to be effective but watch CK. Journal of Pediatrics 149:537-541
Usually <3yr, well, blood culture neg (cf vertebral osteomyelitis - >3yr, toxic, blood culture pos). Rx clindamycin for 2/52 but no evidence! Likely that some of these would resolve spontaneously if untreated.
Adolescents, longbones, probably 1% of all osteomyelitis. ?mech - biopsies usually negative on culture! Pain, ?arthritis if adjacent to focus. Associated with:
ie inflammatory, autoimmune sounding. Osteolytic & sclerotic lesions on XR, hotspots on bone scan. Histology not characteristic. Differential diagnosis is Langerhans cell histiocytosis.
Rx Nsaids, steroids if severe/recurrent, ?pamidronate, ?infliximab. Self limiting but relapsing over 2-4 yrs. 1 case series showed high rate of deformity, but the largest found no long term complications at all!
Neonatal onset multisystem inflammatory disease (NOMID, or Chronic Neurologic Cutaneous and Articular Syndrome ie CINCA) is a rare genetic periodic fever syndrome which causes uncontrolled inflammation in multiple parts of the body starting in the newborn period. Symptoms include skin rashes, severe arthritis, and chronic meningitis leading to neurologic damage. NOMID can result from a mutation in the CIAS1 gene, which helps control inflammation. Mutations in this gene also cause familial cold urticaria and Muckle-Wells syndrome (periodic fever, urticaria, deafness, amyloidosis). NOMID has been successfully treated with the drug Anakinra. (Wikipedia)
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