Paediatric Rheumatology PDF

Paediatrics Updated: Feb 2023 PAEDIATRIC RHEUMATOLOGY JUVENILE IDIOPATHIC ARTHRITIS (JIA) This is the most common form of arthritis in children – usually they are 8 years...

Paediatrics Updated: Feb 2023 PAEDIATRIC RHEUMATOLOGY JUVENILE IDIOPATHIC ARTHRITIS (JIA) This is the most common form of arthritis in children – usually they are 8 years RF: -ve RF: -ve RF: -ve ANA: +ve in 25% ANA: +ve in 75% ANA: -ve ANA: +ve in 70% ANA: -ve CBC: Anemia  Paediatrics Updated: Feb 2023 TREATMENT FOR JIA Generally: Systemic form: Oligoarthritis: Polyarthritis: 1. NSAIDs 1. NSAIDs 1. NSAIDs NSAIDs/steroids→ steroids/DMARDs→ Biological 2. Steroids - pulse therapy 2. Steroids into joints2. Methotrexate(standard dose → higher dose) 3. Methotrexate (DMARD) 3. Hydroxychloroquine 3. TNF blockers 4. Cyclosporine/Azathioprine 4. Methotrexate/ azathioprine 4. Systemic steroids 5. TNF blockers /anakinra 5. TNF blockers 5. Cyclosporin/Azathioprine 6. Cyclophosphamide 6. Cyclophosphamide 7. Stem cell transplant 7. Stem cell transplant  Paediatrics Updated: Feb 2023 JUVENILE SEPTIC ARTHRITIS ETIOLOGY PATHOGENESIS DIAGNOSIS TREATMENT Joint inflammation caused by Toxins of microbes (e.g. ✓ Joint pain Antibiotics microbe (mostly s. caureus). clostricclium extoni) destroy ✓ Redness and swelling Arthrocentesis articular cartilage. ✓ Impaired range of motion or arthrotomy( All joints can get affected e.g. ✓ Fever wash out) synovial joint PAMP (pathogen associated ✓ Symptoms SPECIFIC to the molecular patterns) bacteria Spread o Antigens which are Microbes through preexisting recognized by immune cells infection (e.g. bone) leads to inflammation (due to cytokines). Through blood Fluid accumulates in joint space- Through wound (from high intra-articular pressure environment) Synovial fluid aspiration + test for which leads to compression of WBC, culture blood vessels = necrosis of X-ray/US/CT/MRI for bone affected bones/cartilage erosion/joint effusion especially for WP/ sacroicial- difficult to aspirate.  Paediatrics Updated: Feb 2023 JUVENILE ANKYLOSING SPONDILITIS ETIOLOGY SYMPTOMS DIAGNOSIS TREATMENT Chronic inflammatory Onset is gradual (>3 mo) Genetic: HLA-B27 test 1st line disorder of the sacroiliac Blood test: high CRP/ESR NSADS- ibuprofen or Dull pain: low back, joint and spine. XRAY: sacrolitis grade >2 bilateral OR other analgetic if sacroiliac ( worse when grade 2 or 4 unilateral: contradicted (codeine rest, better after exercise) Epidemiology – males> paracetamol) Pain in the knees, heels, Early; bone Late: females. Late teens- 20s erosions, widening syndesmophyytes Local corticosteroid- and feet that worsens with of sI joints, (vertical for flares Pathogenesis – genetic ( activity romanus symentrical 2nd line HLA- B27), environment Stiffness: last > 30 mins in (vertebral bodies calcification inside Mainly affects sacroiliac the morning, happening for appear square ligament) forming Biologics (anti tnp joints and vertebral > 6months pain as flares) with shiny corners) BAMBOO SPINE alpha)- for high disease activity e.g. column Systemic- weight loss, US – enthesitis diagnosis infliximab fatigue MRI- useful in case of early diagnosis( Surgery- arthroplasty Swelling in the joints of the bone marrow edema) (in case of disability or arms and legs ineffective Chest pain ,enthesitis, medication) SACROLITIS GRADING dactilytis i. Grade 0; normal Physiotherapy Anemia ii. Grade i-ii; mild sclerosis Uveitis (eye pain, redness) iii. Grde iii; widening of joint space iv. Grade iv; bilateral ankylosis Can be associated with IBD  Paediatrics Updated: Feb 2023 RHEUMATIC FEVER (RF) Multisystemic, autoimmune, inflammatory disease of connective tissue that can cause carditis ETIOLOGY DIAGNOSTICS TREATMENT Caused by an abnormal Diagnostic Criteria: Basic steps: autoimmune reaction to a 2 major criteria OR 1. Treat the strep infection (antibiotics) streptococcal infection in 1 major + 2 minor criteria. + Streptococcal 2. Anti-inflammatory meds (steroids) URT. infection (ASO, Culture) 3. Treatment for symptoms (like chorea) 5-15yrs most commonly F:M 3:1 Major Criteria (JONES) Minor Criteria (CAFÉ) 4. Bed rest On average lasts 4 1. Carditis 1. Fever months (4 weeks-2 years) 2. Migratory polyarthritis 2. Arthralgia Antibiotics: Penicillin 14 days 3. Erythema marginatum 3. Elevated acute phase For carditis: For Chorea: 4. Chorea reactants (CRP, ESR) Aspirin 80- Haloperidol 0.5mg/kg/d PATHOGENESIS 5. Subcutaneous nodules 4. Prolonged PR interval 100mg/kg Valproatine acid 15- HLA genetic predisposition 1,2→ Early Symptoms on ECG ↓ Prednisolone 20mg/kg/d 3-5→ Late Symptoms Antibodies against Strep M 1mg/kg Carbamazepine, proteins Treat for 8-12 weeks Phenobarbital 5-7mg/kg/d DDX: ↓ Predominant Arthritis Predominant Carditis Cross reaction with human o Infective arthritis o Infective endocarditis tissues e.g. cardiac muscle, o Reactive streptococcal o Mitral valve prolapse PROPHYLAXIS: skin, brain, SM arthritis o Congenital heart o Quickly and correctly treating strep throat with ↓ o Systemic CT disease defect antibiotics! Type III hypersensitivity o Lyme disease Viral/Contagious reaction Podagra myocarditis, pericarditis  Paediatrics Updated: Feb 2023 SYSTEMIC DISORDERS – SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) ETIOLOGY DIAGNOSTICS A global loss of self-tolerance with activation of >/= 4 criteria (at least 1 clinical and 1 laboratory criteria) autoreactive T and B cells leading to production OR of pathogenic autoantibodies and tissue injury. Biopsy proven lupus nephritis + positive ANA or anti-DNA Clinical Criteria Immunological Criteria Due to: General complaints: Renal Involvement CNS involvement. o Increased ESR, CRP. 1. Innate 2. Environmental o Fatigue o Glomerulonephritis o Seizures o Decreased C3 or C4 Susceptibility Stimuli o Anorexia o Arterial o Psychosis o Positive ANA or o HLA type o UV exposure o Weight loss hypertension o Stroke antiDNR antibodies o Immunoregulatory o Microbial o Persistent fever o Nephrotic o Cerebrospinal genes response o Lymphadenopathy syndrome o Complement o Drugs o Renal failure levels Musculoskeletal Lung damage Skin: o Hormone levels System: o Pleuritis o Butterfly rash on o Arthralgia o Bleeding in lungs. face o Arthritis (2 or DISEASE IN CHILDREN: more joints) o High disease activity onset Cardiovascular: Hematopoietic system o Aggressive clinical course o Pericarditis o Autoimmune o Systematization (rarely isolated form) haemolytic anaemia o Livedo reticularis o Require aggressive treatment. o Thrombocytopenia on extremities, o Leukopenia waist, vasculitis TREATMENT Prevention: Limit severity: Rashes: Joint pain/stiffness: o Limit sunlight o Corticosteroids- To minimize o Immunosuppressants- For o Steroid creams o NSAIDS immune response more severe cases.  Paediatrics Updated: Feb 2023 SYSTEMIC DISORDERS – DERMATOMYOSITIS ETIOLOGY DIAGNOSTICS Inflammation of striated muscle with DIAGNOSTIC CRITERIA: skin involvement. Definite dermatomyositis No.5 + three items from No. 1-4 Probable dermatomyositis No.5 + two items from No. 1-4 Due to: Innate Susceptibility. Possible dermatomyositis No.5 + one items from No. 1-4 o HLA type o Cytokine polymorphism Clinical Criteria 1. Symmetrical, 2. Characteristic triad of myositis 3. Enzyme elevations in blood serum Environmental Stimuli. progressive muscle electromyogram: weakness I. Creatine Kinase o UV exposure I. Short- term, small low-amplitude II. Aldolase polyphasic potentials III. Lactate dehydrogenase II. Fibrillation potentials ,even IV. Transaminase TREATMENT during sleep (ALT/SGPT,AST/SGOT) Corticosteroids: III. Recurring distorted high- o Prednisone high dose frequency discharge Immunosuppressants/Immune 4. Chronic 5. Rashes typical to dermatomyositis inflammation of modulators: muscle in biopsy I. Flaky, scaly rash over II. periorbital III. Flaky, scaly rash in faces, o Azathioprine metacarpal, proximal violaceous neck, extensor surface of o Methotrexate I. Necrosis of muscle interphalangeal joints (heliotropic) extremity particularly in fibre types I and II Physical therapy (Gottrono papules) erythema the hands II. Muscle cell Sunscreen for prevention degeneration and regeneration, different contents of fibre size  Paediatrics Updated: Feb 2023 SYSTEMIC DISORDERS – SCLERODERMA / SYSTEMIC SCLEROSIS CLASSIFICATION DIAGNOSTICS Autoimmune DIAGNOSTIC CRITERIA: disorder where 1 major and 2 minor with age of onset /=3 of the following 10: vasculitis Livedo reticularis (mottled skin) Blood: 1. Weight loss > 4 kg. (not due to dieting/other) Anaemia Systemic Tender nodules 2. Myalgia, leg weakness, foot pain Leucocytosis necrotizing Purpura. 3. Livedo reticularis (mottled reticular pattern over skin) Increased ESR inflammation of Hypergamma- 4. Mono neuropathy, poly neuropathy blood vessels globulinemia 5. Increased urea nitrogen and creatinine Evidence for 6. Increased diastolic blood pressure > 90 mmHg immune complex– Urinalysis: 7. Testicular pain and tenderness (unknown cause) induced disease is Proteinuria 8. Elevated hepatic enzymes suggest hepatitis B infection confined to HBV- Visceral PAN (classic polyarteritis) 9. Angiographic findings (aneurysmal dilatation or segmental related PAN. Kidneys- acute renal failure, stenosis) HTN 10. Biopsy : polymorphonuclear (granulocytes) accumulation of small and medium-sized vessels Brain- headache, CVA Mesentery- abdominal pain. TREATMENT Pulse therapy IV Methylprednisolone