APPROACH TO JOINT PAIN

- Sujay Bhirud 2nd year resident M P Shah Medical College Jamnagar.

CASE 1• An 11 year old boy presents with fever, pain and swelling in

joints, along with shortness of breath. The fever comes and goes at random times of the day. The symptoms have been present now for 4 days. Two days ago, his right knee was painful and swollen, but today it has improved. The joints involved today include the right ankle and left knee. They are quite tender, painful and also swollen. The shortness of breath occurs with walking, but he is now unable to walk because of the joint pain. He also has some shortness of breath with lying down flat when he is trying to sleep.

• Past h/o sore throat 4 wks back which responded to symptomatic treatment.

• T- 38.2, P 160, RR 32, BP 100/60, SpO2 94% in room air. He is tired appearing with tachypnea and tachycardia with a holosystolic murmur 3/6 heard at apex with radiation to axilla. His left knee is swollen and extremely tender with warmth. He has difficulty with range of motion but can flex his knee 30 degrees passively. His right ankle is very swollen and warm. He has limited subtalar motion. Both his knee and ankle are very tender even to touch.

• ESR 110, CRP 9.5, and a chest x-ray with cardiomegaly present. ECG reveals a prolonged PR interval. ASO titer is 754 and streptozyme is 1:600.

• An 11 year old boy presents with fever, joint pain and swelling, along with shortness of breath. The fever comes and goes at random times of the day. The symptoms have been present now for 4 days. Two days ago, his right knee was painful and swollen, but today it has improved. The joints involved today include the right ankle and left knee. They are quite tender, painful and also swollen. The shortness of breath occurs with walking, but he is now unable to walk because of the joint pain. He also has some shortness of breath with lying down flat when he is trying to sleep.

• Past h/o sore throat which responded to symptomatic treatment.

• T- 38.2, P 160, RR 32, BP 100/60, SpO2 94% in room air. He is tired appearing with tachypnea and tachycardia with a holosystolic murmur 3/6 heard at apex with radiation to axilla. His left knee is swollen and extremely tender with warmth. He has difficulty with range of motion but can flex his knee 30 degrees passively. His right ankle is very swollen and warm. He has limited subtalar motion. Both his knee and ankle are very tender even to touch.

• ESR 110, CRP 9.5, and a chest x-ray with cardiomegaly present. ECG reveals a prolonged PR interval. ASO titer is 754 and streptozyme is 1:600.

• Diagnosis of acute rheumatic fever.

CASE 2• A 5yr old boy presented with h/o recurrent pain in joints for last

10 months. It started with fever and pain in left shoulder 10 months back. Was diagnosed as acute septic arthritis and treated with antibiotics for a week. He improved completely within 4-5 days and was well for next 2 weeks. A similar episode occurred again a couple of months later this time involving the Rt wrist and Lt ankle, diagnosed as reactive arthritis and treated with NSAIDs – improved over a week. After an interval of 2 months he developed acute pain in right hip joint and was investigated.

• Lab results: Hb 8%, microcytic hypochromic anemia, WBC 6500/cumm, ESR 25mm, rest tests normal.

• X ray right hip showed an osteolytic area at upper end of femur. Similar area at the lower end of tibia on left side.

• No h/o similar disease or any other complaints prior to onset of these complaints.

• No relevant family history.

• O/E: Poorly built and nourished, moderate pallor, no LN pathy, joints normal, no restriction of movements. Soft tissue swelling over left ankle and right wrist but free from joints. Mild tenderness present, not warm or inflamed.

• Liver 3cm firm nontender. Spleen 2cm firm.• Other system examination normal.

• Hb electrophoresis : Hbs 60%.• Diagnosis: sickle cell disease.

ARTHRITIS is defined by : Intraarticular swelling Or Two or more of the following signs 1. Limitation in range of motion 2. Tenderness or pain on motion 3.Warmth.

ARTHRALGIA: pain without swelling.

ETIOLOGY• Infection : arthritis(viral, septic, tubercular), osteomyelitis.• Trauma• Acute rheumatic fever• Rheumatological disorders: juvenile idiopathic arthritis(JIA), systemic lupus

erythematosus(SLE), dermatositosis, vasculitis, Kawasaki disease, Henoch-Schonlein Purpura(HSP).

• Scurvy• Blood diseases : sickle cell anaemia, hemophilia, leukemia• Reactive arthritis• Malignancies• Psychogenic

WHAT TO ASK?

• Mode of onset and duration of joint involvement.• History of preceding events.• Whether there I involvement of single or multiple joint.• History of trauma, illness or drug intake.• History of fever or other systemic manifestation.• Relationship of pain to activity and sleep.• History of bleeding diathesis.• Is the child suffering from any blood disease?• Dietary history.

Mode of onset and duration of joint involvement.

• Acute : < 2 weeks.• Subacute : 2–6 weeks.• Chronic : > weeks.

• Acute arthritis: ARF, HSP, trauma, transient.• Subacute onset: reactive arthritis, SLE,

Dermatomyositis, PAN, leukemia, sickle cell disease, hemartrosis.

• Chronic arthritis: JIA, Tubercular, psoriasis.

HISTORY OF PRECEDING EVENTS

• Sore throat or Scarlet fever: Rheumatic fever.• GI Symptoms: Reactive arthritis, GI infections like Salmonella or IBD.• Urethritis: Rriter’s disease.• Viral infections [Rubella, mumps, chickenpox, IM, Hep B] ]: Monoarticular synovitis.• Recent immunization (Rubella)• Trauma : Septic arthritis, hemarthosis, effusion, strained ligaments, sprained

muscles, dislocation or fractures.• RTI : Transient synovitis[self limiting, characterized by sudden onset pain in hips,

thigh and knees.]• Pyoderma: Septic arthritis.• H/o drugs[penicllins, immunoglobulins, antitoxins] : Arthritis/Arthragia.

JOINT INVOLVEMENT

• Single joint : Tubercular, septic, momoarticular JIA, trauma.• Multiple joints : systemic disease.[Migratory in rheumatic fever]• Small joints : JIA, Sickle cell disease(hand-foot syndrome), psoriatic arthritis,

tubercular dactylitis.• Large joints : most of the conditions.

H/O FEVER

• High grade fever with chills and joint swelling : Septic arthritis• Long standing, low grade and continuous fever : TB• Prolonged high grade irregular fever with remissions: idiopathic arthritis or

SLE.• Prolonged fever with joint involvement: Luekemias.

PAIN• Location: whether pain is localized to a particular segment or involves a larger

area.• Intensity: usually on a pain scale of 1 to 10.• Onset: was it acute and related to specific trauma or was it insidious? Acute

pain and history of trauma are more commonly associated with fractures.• Pain that worsens on activity : destructive joint pain.• Pain with morning stiffness that improves with activity : JIA.• Growing pains: B/L improves with massage and disappear in the morning.• Joint pain of arthritis worsens on massage• Pain in infection has no diurnal variation. • Fliiting pain : acute rheumatic fever.• Gait and posture: disturbances associated with pain.

• When the sacroiliac or other axial joints are involved, child may experience inflammatory back pain and alternating buttock pain.

• Symptoms characteristic of inflammatory back pain:1. Pain at night with morning stiffness & improving upon rising.2. No improvement with rest3. Improvement with exercise4. Insidious onset5. Good response to NSAIDs

H/O BLEEDING• Bleeding from gums: Scurvy.• Bleeding with hemarthrosis on trivial injury : Hemophilia.• Bleeding PR : HSP.

H/O BLOOD DISEASES : Sickle cell anemia, Leukemia, Hemophilia are associated with joint involvement.

CARDIAC SYMPTOMS: exertional dyspnea, chest pain, palpitation: Rheumatic carditis.

EXAMINATION• Local examination of joints.• Examination of spine.• Examination of muscles.• Presence of rash/ subcutaneous nodules.• Lymphadenopathy/ sinuses.• Eyes.• Oral cavity.• Desquamation of fingers.• Focus of infection.• Organomegaly• Cardiovascular system.

INSPECTION• How the patient moves about in the room before and during the examination as well as

during various maneuvers.• Balance, posture, and gait pattern.• Skin rashes, café-au-lait spots, hairy patches, dimples, cysts, tuft of hair, or evidence of

spinal midline defects.• General body habitus, including signs of cachexia, pallor, and nutritional deficiencies.• Obvious spinal asymmetry, axial or appendicular deformities, trunk decompensation, and

evidence of muscle spasm or contractures.• The forward bending test is valuable in assessing asymmetry and movement of the spine.• Any discrepancies in limb length• Muscle atrophy.• Range of motion of all joints, their stability, and any evidence of hyperlaxity.

PALPATION

• Local temperature• Tenderness• Assessment for a swelling or mass• Spasticity• Contracture• Bone or joint deformity• Evaluation of anatomic axis of limb• Limb length.

EXAMINATION OF JOINTS

• Joints to be examined for : Swelling, pain, tenderness and range of movements.• If it is arthralgia or arthritis.• Single joint is affected or multiple.• Large joint affected in lower extremities, i.e. knee, ankle : oligoarticular JIA.• Small joints of upper and lower extremities : polyarticular JIA.• Spindle shaped fingers : Rheumatoid Arthritis.• Diffuse swelling of entire dorsum of hand and foot : Sickle cell disease.• Scorbutic beading of costochondral junction: Scurvy. • In TB hip joint : limb is flexed, abducted and medially rotated.• Pseudoparalysis [inability to move joint due to severe pain] :Septic arthritis.

• Examination of spine for kyphosis or scoliosis : seen in TB spine.

• Examination of muscles : 1. wasting above or below the joint seen in idiopathic arthritis or chronic joint involvement[Disuse atrophy] 2. tenderness of muscles : Dermatomyositis

Skin rash• Acute Rheumatic Fever : Erythema marginatum.• sJIA : Faint evanescent macular rash[salmon colored] is classic for sJIA. These

are linear or circular and are most commonly distributed over the trunk and proximal extremities, nonpruritic andmigratory with lesions lasting for <1hr. Koebners phenomenon is also present.

• SLE : Typical butterfly shaped malar rash that spares nasolabial fold.• HSP : Palpable purpuric rash over the extensor aspect of extremities.• Juvenile Dermatomyositis : Heliotrope rash over upper eyelids.• Leukemia : Purpuric and ecchymotic patches.• Rheumatic fever : Subcutaneous nodules over extensor aspect of upper

extremities and suboccipital region.

EYES

• Pallor and jaundice with symmetrical painful swelling of hand and feet [Hand-foot syndrome] : Sickle cell disease.

• Iridocyclitis : JIA, AS.• High fever with conjunctival injection : Kawasaki disease.

• Oral cavity : Spongy gums – Scurvy Swollen tongue and lips – Kawasaki disease.• Desquamation of fingers : Kawasaki disease.• Organomegaly : Hepatosplenomegaly : collagen diseases, leukemias, disseminated TB. Splenomegaly : Scikle cell disease.

• Focus of infection viz boils, abscesses, etc: could lead to septic arthritis

CVS

• Hypertension : SLE• Pericardia rub/ Pericarditis: Rheumatoid arthritis, SLE• Myocarditis: Rheumatoi arthritis and Kawasaki disease.

pGALS• Evidence based screening for assessment of school aged children based on

the adult GALS (gait, arms, legs, spine) screen

• Validated with excellent sensitivity and specificity

• Basic clinical maneuvers completed in an average of 2 minutes

SCREENING QUESTIONS

1. Do you have any pain or stiffness in your joints, muscles, or back? 2. Do you have any difficulty getting yourself dressed without any help? 3. Do you have any difficulty going up and down stairs?

GAIT

Observe the child walking

ARMS

Contd..

LEGS

SPINE

DOCUMENTATION OF pGALS SCREENpGALS Screening Questions

Any Pain? Right kneeAny Difficulty Dressing? No

Any Difficulty Walking? Yes

Appearance

Movement

Gait Normal Arms Normal Normal

Legs Abnormal Abnormal

Spine Normal Normal

INVESTIGATIONS

• CBC• ESR• Coagulation factors• Mantoux test• Rheumatoid factor• Antibodies ANA, Anti-DNA• ASO titer• Synovial fluid culture

CBC• In Sickle cell disease and leukemia HB would be low.• Septic arthritis – leukocytosis with polymorphonuclear

predominance• SLE – Leukopenia with Lymphopenia.• Reticulocyte count – increased in Sickle cell disease.• Thrombocytopenia – Leukemia.• Thrombocytosis – Idiopathic arthritis.• HSP – Platelet count will be normal.• Anaemia of chronic disease - Collagen vascular diseases, TB.

• ESR : Increased in collagen diseases and acute rheumatic fever. Decreased in Sickle cell anemia.

• Estimation of clotting factors: when there is hemarthrosis with prolonged clotting time.

• Mantoux test: in suspected case of TB.

• X-ray: changes of Scurvy, TB, Idiopathic arthritis.

• RA Factor : in Polyarticular JIA.• ANA seropositivity: increased risk of chronic uveitis in JIA.

• Anti dsDNA: highly specific of SLE.• ASO Titer: rising titer in rheumatic arthritis indicates recent streptococcal infection.

• CRP: raised in acute rheumatic arthritis.

• Throat swab culture: for isolation of beta hemolytic streptococci in rheumatic arthritis.

• Urine examination: for albumin and hematuria in cases of collagen disease.

• Synovial fluid aspiration: microscopy and culture in septic arthritis.

• USG and MRI: in septic and tubercular arthritis, hemarthrosis.

CATEGORIZATION OF SYNOVIAL FLUID

MANAGEMENT

• Education.• Physiotherapy: -Develop exercise programs -Strengthen muscles & keep joints flexible -Encourage normal limb development -Maintain function and prevent deformities• Analgesics and/or anti-inflammatory drugs• Infection: Antibiotics.• Hemarthrosis: Joint aspiration

THERAPEUTICS

NSAIDS

• Decrease both the pain and acute and chronic inflammation.• But are not disease modifying.

DMARDS

• Biologic and nonbiologic.• Directly affect immune

system.• Steroid sparing agents.

All therapeutic agents suppress inflammation.

• Live vaccines are contraindicated in pts taking immunosuppressive glucocorticoids or DMARDs.

• Before starting steroids or DMARDs : -Negative test result for TB should be verified. -Pts immunization status should be updated.

NSAIDS• Antiinflammatrory effect require regular administration at adequate doses

based on Wt or BSA for longer periods than needed for analgesia alone.• Inhibit enzyme (COX) which is critical in production of prostaglandins.

• Non-selective NSAIDs• Aspirin, Etodolac, Ibuprofen, Naproxen,• Naproxen [tablets and suspension]

• Indicated for patients 2 years and older with juvenile arthritis.• Daily dose: approximately 10 mg/kg/day as a bid dose (5 mg/kg given

twice-a-day). Total daily dose is not to exceed 15 mg/kg/day.

• Selective NSAIDs/cox-2 selective inhibitors• Meloxicam [tablets and suspension]

• Indicated for the relief of the signs and symptoms of oligoarticular and polyarticular course JIA in patients 2 yrs and older.

• 0.125 mg/kg once daily up to a maximum of 7.5 mg.

DMARDs NONBIOLOGIC• Methotrexate• Hydroxychloroquine• Leflunomide• Sulfasalazine• Mycofenolate mofetil• Glucocorticoids -Prednisolone -Methylprednisolne -Triamcinolone

BIOLOGIC• TNF-a antagonists -Adalimumab -Etanercept -Infiximab• T-Cell modulator – Abatacept• IL-1 antagonist – Anakinra - Canakinumab• IL-6 antagonist –Tocilizumab• IVIG

METHOTREXATE• An antimetabolite• MOA: inhibition of folate dependent process increase in

extracellular adenosine increase camp inhibit tnf-a and il-1b inhibit lymphocyte activation and proliferartion.

• Indication: poliarticular JIA, JDM, SLE.• Dose: 10mg/m2 once a wk.• Toxicity: elevated liver enzymes, GItoxicity, headache, alopecia,

leucopenia.• Folic acid 1mg daily given to minimize adverse effects.

GLUCOCORTICOIDS• Oral, IV, Ocular, Topical, Intraarticular.• Oral steroids are foundational treatment of moderate to

severe systemic diseases: SLE JDM.• IV : To treat severe acute manifestations of systemic

diseases; higher doses provide immediate and profound anti-inflammatory effect. Methyprednisolone[10-30mg/kg/dose max 1gm over 1hr for 1-5 days] is preparation of choice.

• Ocular: Drops or sub-Tenon capsular injestion for acute uveitis.

• Intraarticular steroids: Initial therapy for oligoarticular JIA or as bridge therapy while awaiting efficacy of a DMARD in polyarticularJIA.

• Adverse events: Hypertension, iatrogenic Cushing’s syndrome, growth suppression, fractures, cataracts, increased susceptibility to infection

CASE 3• A 10 yr girl with severe pain & swelling in left knee and ankle joint • Also c/o fever, low grade with no rigors, more in evening since one

month.• c/o feeling of stiffness in these joints after inactivity ,poor sleep

quality. • O/E pallor present, spleen 2cm soft non tender, liver not palpable.• Left knee, left ankle, PIP joint of rt middle and ring finger and left

middle finger were swollen.• Tenderness in these joints present.

Lab results:• Hb 9, WBC 4600, PLT 1.5 lacs, • ESR 25, peripheral smear exam normal, PT,APTT

normal.• CRP 42, S. Feritin 310. • RF negative, ANA positive, Anti DsDNA negative.

JUVENILE IDIOPATHIC ARTHRITIS

• Most common rheumatic disease in children.• The former American College of Rheumatology used the term

Juvenile Rheumatoid Arthritis.• The International League of Associations for

Rheumatology(ILAR) uses the term Juvenile Idiopathic Arthritis.

CRITERIA FOR THE CLASSIFICATION OF JIA

• Age at onset: <16 yr• Arthritis in ≥1 joints• Duration of disease : ≥6 wk• Onset type defined by type of articular involvement in the 1st 6 mo after onset: Polyarthritis: ≥5 inflamed joints Oligoarthritis: ≤4 inflamed joints• Systemic disease: arthritis with rash and a characteristic quotidian fever*• Exclusion of other forms of juvenile arthritis

*Quotidian fever is defined as a fever that rises to 39°c(102.2f) once a day and returns to 37°C(988.6f) between fever peaks.

• Oligoarthriticular is the most common subtype (40-50%), followed by polyarticular (25-30%) and systemic- onset (5-15%).

• There is no sex predominance in systemic onset JIA (sJIA)

• but more girls than boys are affected in : oligoarticular (3 : 1)

Polyarticular (5 : 1)

• The peak age at onset is between 2- 4 yr for oligoarticular disease.

• Age of onset has a bimodal distribution in polyarthritis, with peaks at 2-4 yr and 10-14 yr.

• sJIA occurs throughout childhood withpeak between 1-5 yr.

CLASSIFICATION OF JIA (ILAR)• Oligoarticular JIA arthritis affecting 1-4 joints during the 1st 6 mo of disease. Two subcategories are recognized:1. Persistent oligoarthritis—affecting ≤4 joints throughout the disease course. 2. Extended oligoarthritis- affecting >4 joints after the 1st 6 mo of disease.• Poliarticular JIA (RF positive): arthritis affecting ≥5 joints during the 1st 6

mo of disease; ≥2 tests for RF at least 3 mo apart during the 1st 6 mo of disease are positive.

• Poliarticular JIA (RF negetive): arthritis affecting ≥5 joints during the 1st 6 mo of disease; a test for RF is negative.

• Systemic JIA• Psoriatic arthritis• Enthesitis-related arthritis

ETIOLOGY

• Autoimmune disease in which the cause of arthritis is largely unknown

• Combination of factors-Environment (infection, trauma, stress)-Immuno-genetic

PATHOGENESIS• JIA is an autoimmune disease associated with alterations in both Humoral and cell-mediated immunity. • Recruitment of T lymphocytes specific for synovial non-self

antigens -> Complement consumption, immune complex formation, & B-cell activation also promote inflammation. • sJia is characterized by dysregulation of innate immune system

with a lack of autoregulatory T cell and autoantibodies may be more accurately classified as an Autoinflammatory disorder.

• All these immunologic abnormalities cause inflammatory synovitis.

CLINICAL FEATURES• Arthritis must be present for a diagnosis of any subtype

of JIA.• Easy fatigability.• Poor sleep quality.• Involved joints are often swollen, warm to touch, and

painful on movement or palpation with reduced range of motion

• Discrepancy in limb lengths.

• Oligoarthritis predominantly affects the large joints of the lower extremities, such as the knees and ankles.

• If ANA test is positive there is increased risk for asymptomatic anterior uveitis.

• Rheumatoid nodules are associated with a more severe course and almost exclusively occur in RF-positive individuals.

• Micrognathia reflects chronic temporomandibular joint (TMJ) disease

DIAGNOSIS

• JIA is a clinical diagnosis of exclusion with many mimics and without diagnostic laboratory tests.

• Laboratory studies, including tests for ANA and RF, are only supportive and their results may be normal in patients with JIA.

LAB FINDINGS• Elevated WBC and platelets with microcytic anemia, increased

CRP & ESR reflects degree of systemic or articular inflammation.• Anti CCP antibody, like RF, is a marker of more aggressive

disease.• S.Ferritin: elevated [markedly increased in MAS(>10,000

ng/ml)]• Early radiographic changes - soft tissue swelling, periarticular

osteoporosis, and periosteal new-bone formation.• Characteristic radiographic changes in cervical spine, most

frequently in the neural arch joints at C2-C3 may progress to Atlantoaxial subluxation.

• MRI is more sensitive than radiography to early changes

• EARLY CHAGES OF JIA.

ONGOING ARTHRITIS

This is a typical radiograph of a patient with polyJIA, the right carpal bones are osteopenic, with crowding, joint space narrowing and with erosions. The MCPs and PIPS are swollen, and have some ulnar deviation, and

maybe some subluxation visible.

TREATMENTGOALS:• Achieve remission, prevent or halt joint damage, foster normal growth and

development. • Individualized treatment plan according to disease subtype and severity,

presence of poor prognostic indicators, and response to medication. OLIGOARTHRITIS : NSAIDs [If no response with 4-6wk t/t]

Intraarticular inj. Triamcinolone hexacetonide [If no response]

DMARD(Methotrexate) [No response]

TNF-a inhibitors

• POLYATRTICULAR: Methotrexate may take 6-12 wk to see the effects if failure of monotherapy then Biologic DMARD.[anti TNF-α and IL-1,IL6 inhibitors]• sJIA: Combination of TNf-α blockade and methotrexate• Systemic steroids are recommended only for

management of severe systemic illness, for Bridge therapy during the wait for therapeutic response to a DMARD, and for control of uveitis.

POOR PROGNOSTIC INDICATORS• Oligoarticular JIA: -Extended oligoarticular disease.• -Oligoarticular disease wth ANA positive.• Polyarticular JIA: -Young age at onset,• -RF seropositivity or -Rheumatoid nodules, -Presence of anti-ccp antibodies, -large numbers of affected Joints. -Disease involving the hip and hand and wrist is also associated• sJIA: Polyarticular distribution of arthritis, Fever lasting >3 mo, Increased inflammatory markers[ platelet count and ESR, for >6 mo].

ENTHESITIS RELATED ARTHRITIS

• ERA Is a group of diseases affecting joints of the lower extremities and, eventually, the axial skeleton, & it is characterized by absence of autoantibodies & RF and by a strong association with (HLA-B27).

• SPONDARTHRITIS: Include AS, psoriatic arthritis, UC, CD, juvenile chronic arthritis, Whipple Disease, Behçet syndrome, RA, and acute Anterior uveitis.

• ENTHESIS: Insertion of ligaments and tendons into bone• Asymmetrical arthritis affected 4 or fewer joints• Male predominance

COMMON CHARACTERISTICS:• 1. Inflammation of the joints of the axial skeleton and of entheses is the most important clinical feature

• 2. Relatives of children with JAS or ERA commonly have AS, psoriatic arthritis, IBD, or, less commonly, RA and have high frequency of B27 in these families.

• 3. Share a number of extraarticular features e.G. Uveitis.

• 4. RF and other autoantibodies are absent.

ENTHESITIS-RELATED ARTHRITIS (ILAR CLASSIFICATION)DEFINITION

Arthritis and enthesitis Or

Arthritis or enthesitis with at least two of the following:1. Sacroiliac joint tenderness and/or inflammatory spinal pain2. Presence of HLA-B273. Family history in at least one first- or second-degree relative with medically confirmed hla-b27-associated disease4. Anterior uveitis that is usually associated with pain, redness, or photophobia5. Onset of arthritis in a boy after 8 yr of ageExclusions• Psoriasis confirmed by a dermatologist in at least one first or second-degree relative• Presence of systemic arthritis

JUVENILE ANKYLOSING SPONDYLITIS

• Enthesitis of axial skeleton and sacroiliac joints. • Present with back pain• Loss of lumbosacral mobility

• Oligoarthritis of joints of lower extremities• Common presentation

Male with back pain, morning stiffness that is relieved with exercise

• Labs • HLA-B27 positive• Increased ESR• ANA and RF are NEGATIVE

• Radiology• Bamboo spine

• Treatment• NSAIDS, sulfasalazine, mtx

PSORIATIC ARTHRITIS• 10% of JIA.• Definition[ILAR]: Arthritis and psoriasis, or arthritis and at least 2 of

the following: 1. Dactylitis. 2. Nail pitting and onycholysis. 3. Psoriasis in a 1st-degree relative• Bimodal: preschool and early adolescent. M=F• Preschool: M>F, ANA+, increased risk of asymptomatic ocular

inflammation.• Asymmetric arthritis with 4 or less joints at presentation.• Knee, ankle, small joints(finger, toes).• DIP joint involvement is uncommon but highly suggestive of

diagnosis.

CASE 4• A 8-year-old boy c/o severe pain in b/l wrist and ankle joint.• h/o fever, diarrhea, and abdominal pain for 5 days. A week

later he developed arthralgia of the bilateral sacroiliac, wrist, and ankle joints.

• O/A: T normal, P 115, and BP 110/70. • O/E swelling and tenderness of left ankle joint and, tenderness on motion and pain over the bilateral wrist and left sacroiliac joints

Lab results:• Hb12.8 g/dl, WBC 21,860/mm3, PLT 573,000/mm3, ESR 74 mm/hr,

CRP 3.97. • ANA negetive. Urinalysis revealed pyuria, but culture was negative. • HLA B27positive. • Aspirate revealed severe inflammation, but gram staining and

culturing of this synovial fluid gave negative results. • The stool culture was positive for salmonella.• Xray only soft tissue swelling, w/o any s/o of enthesopathic lesions

or jt damage• Treated with NSAID (naproxen, 500 mg every 12 hour) for 7 days,

but there was partial improvement. So added prednisolone (10 mg every 8 hour).

REACTIVE AND POSTINFECTIOUS ARTHRITIS

Postinfectious arthritis

• Arthritis that occurs after group A streptococcus or viruses.

• Pain or joint swelling is usually transient, lasting less than 6 wk, and does not share the typical spondyloarthritis pattern.

• Reactive arthritis• Refer to arthritis that occurs following

enteropathic or urogenital infections• The course is variable and may remit

or progress to a chronic spondyloarthritis including ankylosing spondylitis.

• 75% HLA B27 positive.

Defined as joint inflammation due to a sterile inflammatory reaction following a recent infection.

• REACTIVE ARTHRITIS

• Enteric infection: salmonella, shigella, yersinia enterocolitica, campylobacter jejuni, cryptosporidium parvum, or giardia intestinalis.

• Genitourinary tract infection with chlamydia trachomatis or ureaplasma.

• Rubella• Chikungunya• Parvoviruses• Adenovirus 7• Herpesviruses:• Epstein-barr• Cytomegalovirus• Varicella-zoster• Herpes simplex

• Mumps• Hepatitis B• Enteroviruses:• Echovirus• Coxsackievirus B

POSTINFECTIOUS ARTHRITIS

CLINICAL FEATURES

• Symptoms: present 2-4 wk following infection. • The classic triad of arthritis, urethritis, and

conjunctivitis (formerly referred to as reiter syndrome) is relatively uncommon in children.

• The arthritis is typically oligoarticular, with a lower extremity predilection.

• Dactylitis and enthesitis is common

• Cutaneous manifestations: circinate balanitis, ulcerative vulvitis, oral lesions, and keratoderma blennorrhagica.

• Systemic symptoms: fever, malaise & Fatigue. • Early in the disease course, markers of inflammation—ESR, CRP & platelets may be markedly elevated

DIAGNOSIS: A recent genitourinary or gastrointestinal infection may suggest the, but there is no diagnostic test.

TREATMENT: - Specific treatment is unnecessary for most cases. - Nsaids are often needed for management of pain and functional limitation. - Intra-articular steroid injections may be utilized for refractory or severely - Involved joints once acute infection has been ruledout. - Systemic steroids or DMARDS are rarely needed

CASE 5• A 2 yr/m child with fever and refusal to walk for two

days.• c/o pain and points to his right lower extremity.• There is a recent history of an URTI about two weeks

ago, but no recent trauma. • The pain is not known to migrate.

Exam: • T 39.5, P 120, R 18, BP 100/50, SpO2100% in RA.• Wt 10%ile, ht 50%ile. • He is thin appearing and refuses to walk. He is not fussy

and nontoxic.• lying in a hospital bed with his right lower extremity

externally rotated, abducted, and motionless. • He has severe discomfort with minimal internal and

external rotation of the right hip despite attempts to distract him.

Laboratory studies:• wbc 20,000, 8% lymphs, 2% monos, platelet count

265,000. ESR 45. CRP 12. Hip radiographs show widening of the acetabular space on the right.

• An orthopedic surgeon is consulted. An arthrocentesis of the right hip is performed which shows WBC 110,000, glucose 35, gram stain shows many wbcs and few gram positive cocci.

• Surgical debridement of the right hip is performed. Empiric treatment with vancomycin and ceftriaxone is initiated after cultures are obtained. Culture shows staph aureus sensitive to methicillin.

• Vancomycin and ceftriaxone are discontinued and the patient is treated with oxacillin.

• Within three days of treatment onset, his fever declines and he slowly begins to ambulate..

SEPTIC ARTHRITIS

• An infection of the joint space• Monoarticular-90%, polyarticular10%.

• Septic arthritis is more common in young children. • 50% cases occur by 2 yr of age and 75% of all cases occur by 5

yr.

ROUTES OF INFECTION

1. Hematogenous• Most common• Bacteremia associated with URTI, skin or GIT infections. or invasive procedures.

2. Direct inoculation• Joint contamination by foreign object

3. Contiguous spread• Osteomyelitis due to several factors in infants

ETIOLOGY

• Staphylococcus aureus is now the most common infection in all age groups.

• But historically, h. Influenzae type b accounted for more than half of all cases of bacterial arthritis in infants and young children.

• S. Pneumonia• N. Meningitidis• Group b streptococcus.• Gonococcus – in adolescents and neonates.

Risk factors• Neonates• Umbilical vessel catheterisation• Central venous catheters• Femoral vessel blood sampling• Osteomyelitis• Immunodeficiency• Joint surgery• Thalassemias• Diabetes

PATHOGENESIS• Mostly hematogenous seeding of the synovial space.• Less often, by direct inoculation or extension from a contiguous focus• Synovial and cartilage destruction results from a combination of proteolytic

enzymes and mechanical factors.• Bacterial products stimulates cytokine production (TNF-α, IL-1) triggering

an inflammatory cascade.• Proteolytic enzymes and elastases are released by neutrophils, synovial cells

and chondrocytes cause destruction cartilage and synovium.• Bacterial hyaluronidase breaks down the hyaluronic acid in the synovial fluid,

making the fluid less viscous decreased lubrication increased friction.

• Increased pressure within the joint compromise the vascular supply and induce pressure necrosis of the cartilage.

• Infant: destroy the epiphysis, which is still largely cartilaginous

• Children: vascular occlusion lead to necrosis of epiphyseal bone

HEALINGMay occur with/and/or

1.Complete resolution

2.Partial loss of articular cartilage and fibrosis of joint

3.Loss of articular cartilage and bony ankylosis

4.Bony destruction and permanent deformity

CLINICAL FEATURES

INFANTS• More septicaemia rather than joint pain• Baby is irritable & refuses to feed• Tachycardia with fever• Joints are inflamed• Pseudoparalysis• Check umbilical cord and IV site for infection

Children• Acute pain in single large joint(esp hip)• Pseudoparesis• Child is ill, rapid pulse and swinging fever• Overlying skin looks red & superficial joint swelling

may be obvious • Local warmth and marked tenderness• All movements are restricted by pain or spasm.

PHYSICAL EXAMINATION

• Lower limb antalgic limp / cannot walk• Upper limb affected part is closely guarded• Marked tenderness, active and passive range of motion

are limited• Examine for synovial effusion, erythema, heat and

tenderness.• Spasm of muscles around the joint may be marked.• Patient may hold the joint in a position to reduce the

intra-articular pressure to minimize pain.

INVESTIGATIONS• CBC: increased WBC count.1.ESR and CRP are better negative than positive predictors of sa.

Good for monitering progress.2.ESR : May rise 3-5 days after therapy. Can stay elevated for a

month.3.CRP: Peaks within 30- 48 hours of infection and falls to normal

within a week of treatment4.Other tests are PCR for K. Kingae5.Cultures from genitals if gonococcal suspected6. Gas asot to be done

MANAGEMENT• Medical

• General supportive: control of fever & pain, hydration and splinting affected limb in position of least pressure possibly with traction to prevent dislocation.

• NSAID will also stop PG production thereby reducing cartillage destruction

• Antibiotics: iv antibiotics 6-8 weeks• Surgical

• Percutaneous arthrocentesis• Arthroscopic of open surgical drainage

• Rehabilitation• Physiotherapy: rapid mobilizaton

• Indications for open drainage :

1.Hip septic arthritis2.Long duration3.Exclusion of foreign body4.Large amounts of fibrin5.Debridement in those with osteomyelitis6.Lack of clinical improvement after 48 hours of antibiotic

therapy

MUSCULOSKELETAL PAIN SYNDROMES• Pain complaints of at least 3 mo in duration in the

absence of objective abnormalities on physical examination and laboratory screening and pain persists despite previous treatment with NSAIDs and other analgesic agents.

• Females > Males.• Localised or more diffuse.

• Commonly accompanied by psychologic distress, sleep difficulties, and functional impairment throughout home, school, and peer domains

• May report loneliness and social isolation, characterized by having few friends and lack of participation in extracurricular activities.

• Diagnosis of exclusion

CASE 6• A 7 yr boy came with a one month h/o upper chest and shoulder

pain. • O/E reduced range of movements in his shoulder joints,

restricted by pain• X-ray shoulder joints was normal. ESR 90 mm/hour.• Diagnosed as having musculoskeletal injury and was given

ibuprofen.• A month later with pain in hip, knee, and ankle joints bilaterally.

This time diagnosed as polyarticulat JIA. Bed rest and physiotherapy given & Naproxen.

• A month later had exacerbation - started prednisolone.• Now diagnosed as symmetrical polyarticular arthritis.

Intra-articular steroid injections were given and started methotrexate – improvement

• A month later re-admitted with severe pain and high fever. CBC showed Hb 8.1, PLT 121×109/l, and neutropenia of 0.8×109/l, WBC of 2.8×109/l and presence of blast cells on the peripheral film.

• Bone marrow examination confirmed the diagnosis of acute lymphoblastic leukaemia.

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