Pediatrics2 SE2 High Yield Reviewer (1)

HIGH YIELD PEDIATRICS2 SE2 REVIEWER (PEDIATRIC INFECTIOUS DISEASES) I.

OVERVIEW: PEDIATRICS INFECTIOUS DISEASES • Top 5 leading causes of morbidity: 1. Pneumonia 2. Diarrhea 3. Bronchitis/ Bronchiolitis 4. Influenza 5. Hypertension • Special problems of children: 1. First exposure - exposure to an agent for the first time often produces fever and severe illness; re-exposure likely to produce a mild illness, modified by the serum antibodies from the first infection 2. Small passages - more easily obstructed by edema or secretions 3. Young cells - rapid growth rates of cells make cells more susceptible to infection with most viral agents; decreased interferon production 4. Immature immunologic defenses - IgM are not usually synthesized by the fetus unless exposed to maternal infection; decreased complement activity; decreased neutrophil chemotaxis; less effective cell-mediated immunity • Clinical approach to infectious diseases: 1. Infectious diseases intertwined with microbiology 2. All living cells appear to be parasitized by microbes throughout their lifetimes 3. Pathogenesis both of virulence of infecting microbes and of defense of invaded host 4. In-depth knowledge of the immune system indispensable in understanding the basic nature of management and prevention of human diseases 5. Microbial infection may be responsible for most human diseases • Two approaches to the study: 1. Etiologic agent approach - identify the characteristics of the infecting organism and the disease it may cause 2. Anatomic syndrome approach - can be defined in purely empirical and mutually exclusive terms and a patient's illness often can be classified into a single anatomic syndrome • General approach to the child with possible infection: 1. Detailed history and PE is essential 2. Use of the laboratory - pathogens identified through direct methods, indirect methods and non-specific laboratory indications of infection (elevation of acute-phase reactants: CRP and ESR)

II.

FEVER OF UNKNOWN ORIGIN (FUO) • Fever - symptom and not a disease; elevation of body temperature above normal (37.4C orally or 38C rectally) Physiologic Fever States Pathologic Causes • Digestion • Infection (most common) • Exercise • Inflammation • Ovulation • Neoplasms (hematologic or solid tumors) • Pregnancy • Vaccines • Warm environment • Dehydration • Emotion • Common causes of fever: Major Illnesses Minor Illnesses • Bacterial meningitis • URTI • UTI • Viral exanthema • Pneumonia • Gastroenteritis • Malaria • Mechanisms of a protective effect of fever: o Enhanced neutrophil migration o Increased production of antibacterial substances by neutrophils o Increased production of interferon o Increased anti-viral and anti-tumor activity of interferon o Increased T-cell proliferation o Decreased growth of microorganisms in iron-poor environment • Extreme hyperpyrexia (>41C) and hypothermia: Extreme Pyrexia Hypothermia • Central fevers • Elderly and the newborn • Drug fever • Cold exposure • Heat stroke • Hypothyroidism • HIV • Overwhelming infection • Malignant hyperthermia • Sepsis in CRF • Malignant neuroleptic syndrome • Overzealous treatment with antipyretics • Fever patterns: o Intermittent pattern - exaggerated circadian rhythm thst includes a period of normal temperature on most days; high spikes with return to normal o Remitting pattern - persistent and varies by more than 0.5C for >24 hours; like intermittent but never returns to normal

o Sustained pattern - persistent and does not vary more than 0.5C for >24 hours; like remittent but with less marked swings of temperature o Relapsing pattern - febrile periods that are separated by intervals of normal temperature; seen in malaria § Tertian - 1st and 3rd days (P.vivax) § Quartan - 1st and 4th days (P.malariae) o *Biphasic pattern - seen in dengue o *Stepladder pattern - seen in typhoid/ enteric fever • Classic working definition (FUO): o Continuous fever of at least 3 weeks duration with daily temperature elevation above 38.3C and remaining undiagnosed after 1 week of intensive study in the hospital • Working definition in pediatrics (FUO): o Fever >38C persisting as a predominant feature for more than 7-10 days in a child in whom a careful, thorough history and physical examination and preliminary laboratory data fail to establish a diagnosis • Etiology: 1. Infectious causes - most common 40-50% • Typhoid fever - stepladder pattern; fever will not be shorter than 5 days to a week; Faget sign (pulse fever disproportion) • Differential diagnosis: o Salmonella infection (Typhoid fever) - seriously and obviously ill with no apparent cause; osteomyelitis/ arthritis (infant) o Malaria - history of travel/ residence in endemic areas; enlarged spleen; jaundice (hemolysis of RBCs) o Miliary (Disseminated) TB - #1 in the Philippines; weight loss; anorexia; tuberculin test (+) or (-); sterile pyuria; small discrete (hyperdense) shadows in both lung fields on CXR; millet seeds o Pyelonephritis - CVA/ suprapubic tenderness; crying on passing urine/ straining; passing urine more frequently than usual; incontinence; WBC and/or bacteria in urine on microscopy o Abscess - fever with no obvious focus of infection; local tenderness (suppuration/ inflammation); fluctuant mass (subphrenic/ psoas) o Septicemia - seriously and obviously ill, highly-febrile child with no focus of infection; purpura, petechiae 2. Collagen vascular 10-20% 10% • Juvenile rheumatoid arthritis - most common 3% • SLE 1% • HSP 1% • Vasculitis 3. Malignancies/ neoplasms 5-10% • Occult solid tumors (neuroblastoma - most common, Wilm's tumor, retinoblastoma) • Hematologic tumors (leukemia, lymphoma, Hodgkin's) 4. Miscellaneous 10-15% • Factitious fever • Metabolic • Genetic 5. Unknown/ undiagnosed 20-30% • Adage: o "FUO is more likely to be caused by an unusual presentation of a common disorder than a common manifestation of a rare disorder" • Clues in the history that may localize the infection: Sore throat Streptococcus tonsillitis Cough, rusty sputum Lobar pneumonia Severe joint pain/ swelling Pyogenic arthritis Severe pain in head and back of the neck with stiffness Meningitis Severe pain in a bone Osteomyelitis Tender liver Amoebic liver abscess Viral hepatitis Ill-defined subcutaneous inflammation Cellulitis Pyomyositis Bloody diarrhea Shigella dysentery Campylobacter Chronic Diseases: Congenital heart diseases Cyanotic heart disease Rheumatic fever Shunted hydrocephalus

Complications: Bacterial endocarditis Cerebral abscess Bacterial endocarditis Recurrence of RF Shunt infection Ventriculitis

Septicemia Chronic renal disease UTI Congenital or acquired immunodeficiency Opportunistic organisms, fungi, parasites Recent surgery Concealed abscesses • History: o Thorough history is essential • Physical examination: o Complete, detailed, thorough and diligent o Repeated frequently o Look for focus of infection or findings • Laboratory: o The most broadly-based, highest-yield, and most cost-effective testing is done first o The lowest-yield, most esoteric testing is done last if no diagnosis is made despite persistence of symptoms III.

GRAM (+) BACTERIAL INFECTIONS: STAPHYLOCOCCUS AND STREPTOCOCCUS 1. Staphylococcus species a) S. aureus b) S. epidermidis 2. Streptococcus species a) S. pneumoniae b) Group A Streptococcus

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b.) •

Staphylococcus species Either coagulase (+) S. aureus or coagulase (-) S. epidermidis, S. saprophyticus, S. haemolyticus Part of the normal flora of humans Coagulase (+) - more virulent; coagulase may cause clumping > bigger > lesser chance to be phagocytosed Disease may result from tissue invasion or injury caused by various toxins and enzymes produced by the organisms S. aureus Most common cause of pyogenic infections of the skin and soft tissue Clinical manifestations vary with the location of infection, which is most commonly the skin but may be any tissue More prevalent among person living in low socioeconomic circumstances and those in tropical climates Toxin-mediated diseases o Food poisoning o Staphylococcal scarlet fever o Staphylococcal scalded skin syndrome (SSSS) § Ritter disease § Prodrome of malaise, fever, and irritability associated with exquisite tenderness of the skin or the appearance of generalized erythema § Flaccid blisters and erosions develop § Circumoral erythema or radial crusting and fissuring around the eyes, mouth, and nose (“sunburst pattern”) § Areas of epidermis may separate in response to gentle shear force (Nikolsky sign) o Toxic shock syndrome (TSS) S. epidermidis Coagulase Negative Staphylococci/ CONS o Phlebitis o Cause infections in patients with indwelling foreign devices o Common cause of nosocomial neonatal infection

Streptococcus species

a.) • • • •

b.) • • •

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S. pneumoniae (Pneumococcus) Gram (+), lancet-shaped polysaccharide encapsulated diplococcus Encapsulated strains cause most serious disease Capsular polysaccharides impede phagocytosis Common clinical syndromes: otitis media, sinusitis, pneumonia and sepsis (bacteremia)

Group A Streptococcus (Streptococcus pyogenes) Gram (+) coccoid-shaped bacteria that tend to grow in chains Classified on the basis of the M protein antigen, the virulent factor Also cause distinct clinical entities (scarlet fever and erysipelas, as well as toxic shock syndrome and necrotizing fascitis Pathogenesis: direct invasion and toxin mediation Diagnosis: throat swab culture or rapid antigen

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Impetigo bullosa Exclusively staphylococcal in origin The characteristic lesion are caused by epidermolytic toxin Staphylococcal pneumonia Pneumatoceles – (air-filled) vs. Streptococcus pneumoniae (consolidation) Rare

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detection test Non-specific pharyngotonsillitis - kissing tonsils Highly suggestive of Group A B- Streptococcal infection: palatal petechiae Scarlet fever Toxin-mediated disease Skin has goose-pimple appearance and feels rough Face usually spared, although the cheeks may be erythematous with circumoral pallor After 3-4 days, the rash begins to fade and is followed by desquamation (Measles and Scarlet fever) The tongue is usually coated and the papillae are swollen (strawberry tongue) Impetigo contagiosa The classic lesion begins as erythematous papules in traumatized areas Vesiculopustular lesion Quickly evolve into honey-colored crusted plaques with surrounding erythema GAS and S. aureus – chief causative agents

DISEASES PRESENTING WITH A VESICULOPAPULAR RASH AND PETECHEAL/ PURPURIC ERUPTIONS • Vesicles: measure 5 mm • Varicella (Chicken Pox ) o In normal immunocompetent children, systemic symptoms usually mild, serious complications unusual o Mode of transmission: respiratory route or direct contact with skin lesions of varicella/ herpes zoster patients o characterized by short or absent prodromal period o Characterized as “dewdrops on a rose petal” o Most striking manifestation of lesion: rapidity of progression from macule to papule to vesicle with clouding and umbilication within 24 to 48 hours and ends in crusting o Lesions appear in crops o Papulovesicular eruptions associated with fever and mild constitutional symptoms o Appearance of lesions simultaneously in one anatomic area o Predominant central distribution of lesions including the scalp o Eventual crusting of all skin lesions o Congenital (Fetal) Varicella Syndrome - cicatricial skin lesions; ocular abnormalities; hypoplastic limbs; cortical atrophy o Herpes zoster (Shingles) - varicella zoster virus (VZV) establishes latent infection in dorsal root ganglia and reactivate • Herpes simplex o Herpes simplex type 1 (HSV-1) - associated chiefly with infections of the mouth, lips, eyes and central nervous system (above the waist) o Herpes simplex type 2 (HSV-2) - associated with genital and neonatal infections o HSV-1 - site of latency is the trigeminal ganglia o HSV-2 - site of latency is the sacral ganglia o Viral infection begins at a cutaneous portal of entry such as oral cavity, genital mucosa, conjunctiva or breaks in keratinized epithelia o Acute Herpetic Gingivostomatitis o Herpetic Whitlow • Enteroviral exanthems o Hand-foot-and-mouth: Coxsackievirus A16, also A5, A7, A9, A10, B2, B5 o Herpangina: Coxsackievirus A22, also A1-A10, A16 o Insect bite-like: Coxsackievirus A4; crops, last 1-2 weeks o Non-pustule forming: Coxsackievirus A9; also Echovirus 11, 30 • Erythema multiforme o Erythema multiforme major (Stevens-Johnson Syndrome) § Cutaneous and mucosal lesions have an abrupt onset following a prodromal respiratory illness § Bullae involved the lips, the mouth, and conjunctiva § Purulent conjunctivitis is usual as is uveitis o Erythema multiforme minor § Lesions begin as macules or wheals and evolve into papules or plaques § Center of the lesion may be vesicular, purpuric or necrotic § Iris or target lesions - pathognomonic sign _________________________________________________________________________________________________________________ • Dengue fever o Benign syndrome o Biphasic fever o Headache, myalgia, arthralgia, rash - morbilliform, maculopapular, leukopenia, lymphadenopathy, petecchiae

Phases of dengue: § Febrile phase - flu like symptoms and dehydration § Critical phase - cardiovascular compromise to shock from “leaky” capillaries, thrombocytopenia and coagulopathy § Recovery phase - reabsorption of fluid, may develop hypervolemia in overly aggressive fluid management o Torniquet test: § Positive test: 20 or more petechiae per 1 inch2 (6.25 cm2) o Clinical case definition for Dengue Hemorrhagic Fever: § Fever, or recent history of acute fever § Hemorrhagic manifestations § Low platelet count (100,000/mm3 or less) § Objective evidence of “leaky capillaries” o Complications: § Carditis § Encephalitis § Severe pleural effusion § End organ damage in severe shock - CNS, renal o Dengue Shock Syndrome § Severe protein-losing shock syndrome § Rapid and weak pulse § Narrow pulse pressure ( large confluent ecchymoses that blister, necrose and develop eschar § Purpura fulminans § Waterhouse-Friderichsen Syndrome § Adrenal hemorrhage Congenital Rubella Syndrome o IUGR o “Blueberry muffin spots” o Thrombocytopenic purpura with petechiae o

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V.

COMMUNICABLE VACCINE-PREVENTABLE DISEASES

Vaccine Type: Live attenuated

Live bacterial Killed inactivated Killed bacteria

Subunits

Examples: MMR OPV Varicella Yellow Fever BCG Oral typhoid HepA Polio Rabies Cholera Pertussis Plague Typhoid Acellular pertussis HepaB influenza typhoid

Toxoid Polysaccharide (ineffective in children) Conjugate polysaccharide

lyme Tetanus Diphtheria Meningococcal disease Pneumococcal disease Hib Pneumococcal disease

Vaccine: (Birth to 2 years old) BCG ** Hepa B DTP Hib Polio Pneumococcal conjugate MMR Measles Varicella

Number of doses:

Vaccine: (4 to 6 years old) DTP Polio MMR Hepa A

Number of doses:

1 3 4 4 4** (3 in USA) 4 1 1 1

1 1 1 2

DISEASE

TYPE OF ORGANISM

Diphtheria

Corynebacterium diptheria

Gram (+)

MODE OF TRANSMISSION Person to person, direct contact

Haemophilus influenza B

Gram (-)

Person to person, direct contact or airborne droplets

Hepatitis B

Virus

Exposure to infected blood or body fluids In children, primarily prenatally spread

Mumps

Virus

Pertussis

Bordetella pertussis Gram (-)

Person to person contact or droplet Communicable the day before to 9 days after swelling Most often occurs in children Person to person contact Very contagious

SYMPTOMS

COMPLICATIONS

“Bullneck appearance” (Tonsillar and Pharyngeal Diptheria), sorethroat, fever, yellow-white to grayish membrane tonsils (leathery) Laryngeal diphtheria: brassy or metallic cough Otitis media, sinusitis, epiglotitis, upper respiratory tract infection Generally flu-like symptoms (may be asymptomatic) Liver may be enlarged, dark urine, light stool, jaundice Symptoms last 4-6 weeks Low-grade fever, headache, carache Pain and swelling of parotid glands lasting about a week

Respiratory distress, myocarditis

Non-productive cough with quick expiratory phase followed by inspiratory whoop (“whooping cough”) Scleral or conjunctival hemorrhage due to coughing

Pneumonia, fever, ear infections

ADDITIONAL NOTES

Bacterial meningitis Most invasive disease occur in children 3 mos to 3 y/o Chronic hepatitis, cirrhosis, liver cancer

Orchitis in males who have reached puberty but sterility is rare Infrequent complications are encephalitis and meningitis Leukocytosis/ leukemoid reaction with lymphocytosis

Pneumococcus

Gram (+)

Polio

Polio virus + enterovirus with serotypes 1,2,3 Virus

Tetanus

Clostridium tetani Neurotoxin produced by anaerobe Gram (+)

Influenza

Virus type A or B

Rotavirus

Virus

Fecal-oral route, usually from contaminated water

Human

Virus

Sexually transmitted especially among promiscuous adolescents

Papillomavirus

Person to person contact or droplets Many people are colonized in the upper respiratory tract Direct contact of virus with mouth

Tetanospamin Exposure of wound to the bacterium Affected muscles sustain maximal contraction and annot relax Deep puncture wounds are at greatest risk Not transmissible from person to person Neonatal tetanus results from contamination of the umbilical cord stump Person to person, contact with airborne droplets (most common mode of transmission)

Otitis media, sinusitis, invasive bacterial infections

Pneumonia, meningitis

Low-grade fever and sore throat (most cases are asymptomatic) Differential diagnosis: Assymetric ascending paralysis Occur only in humans “Rope sign” (Paralytic bulbar polio) Severe generalized muscle spasms Trismus (masseter muscle spasm: lock jaw) most common presenting manifestation Rissus sardonicus (sardonic smile of tetanus) - intractable spasms of facial and buccal muscles Opisthotonus – rigidity of abdominal or thoracic muscles Fever, myalgia, headache (flu-like symptoms) are prominent in older children and adults Younger children asymptomatic Epiglotittis Watery diarrhea Child 6 years GBS H. influenza, Type B N. meningitides E.coli S. pneumonia S. pneumonia L. monocytogenes N. meningitides Enterococci S. pneumonia Complications: § Seizures, subdural effusions, SIADH, prolonged fever, DIC o Sequelae: § Cerebral atrophy § Microcephaly o Treatment: § Uncomplicated penicillin sensitive s. pneumonia mengitis: 10 -14 days § Resistant strep: vancomycin § Uncomplicated N. meningitides: IV penicillin 5-7 days § Uncomplicated h. influenza type B: 7-10 days § Gram (-) meningitis: 3rd generation cephalosporin Viral Meningitis o Aseptic meningitis o Usually accompanying the viral infection as diarrhea, respiratory, mumps, measles, varicella o Treatment is supportive as the course is self-limiting TB meningitis o 3 stages: § Stage 1 –prodromal (fever, headache, irritability) § Stage 2 –neurologic signs (meningeal signs, decreased level of sensorium) § Stage 3 –coma, irregular respiration, rigidity, opisthotonus o Diagnosis: § Clinical: subacute course, (+) exposure to TB, (+) tuberculin test § CSF: groundglass appearance with tendency to coagulate, increased WBC with lymphocytes, extremely high protein, low sugar § (+) Acid fast staining, PCR, ELISA, or latex agglutination antigen o

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Normal

Treatment: § Quadruple anti-TB regimen: • 2 HRZES/ 10 HR WBC 0-5 lymphocyte

15-45

Protein

Sugar 50-75 >50% of blood sugar

Clear

Others

ABM

High neutrophils (After 5 days lymphocyte)

High

Low

Turbid

Viral

Normal

N or slight increase

Normal

Clear

High lymphocyte

High

Low 6 months, in at least 2 settings and significantly affects social, academic or occupational functioning •

X.

SEIZURES: •

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Simple Partial Seizures/ Focal Seizures/ Jacksonian Seizures: o Consciousness is preserved o May have motor, sensory, somatosensory or special sensory, autonomic or psychic symptomatology. o No post-ictal confusion Absence o Brief (less than 30 seconds) o With impairment of consciousness o No aura o No post-ictal symptoms o Typical EEG: generalized 3-4 Hz slow spike and wave discharges o Fundamentally and pharmacologically unique from other seizures Myoclonic o Brief jerks of whole body or individual muscle groups, usually without impairment of consciousness o Shock-like contractions are commonly precipitated by the patient’s falling asleep or awakening.

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o May evolve into a tonic-clonic seizure Atonic Seizures: o Sudden loss or diminution of muscle tone without apparent preceding myoclonic or tonic events, lasting for 1-2 seconds involving the head, trunk, jaw or limb musculature

Diagnosis: • • • •

No routine laboratory requests EEG Neuroimaging: MRI>CT Others for specific indications: o CSF, head-up tilt table test and/or other cardiologic work-up o Endocrinological, metabolic, genetic work-up, neurppsychological investigations Use of EEG: • Abnormal epileptiform activities: o Absent 10-40% of epileptic patients o Present in 1-5% on non-epileptic people Indications for Neuroimaging: • Partial onset seizures • Findings suggest of progressive neurologic disease • Intractable seizures • Seizures increasing in frequency and intensity despite adequate treatment. • MRI- has higher sensitivity than CT Scan for structural epileptogenic foci. Infantile Spasm or West Syndrome: • Triad: Infantile spasm, mental retardation and hypsarrythmia on EEG Lennox Gastaut Syndrome: • Triad of seizure disorder, mental retardation and atypical petit mal on EEG • Mixed seizure type Benign Rolandic Epilepsy: • Male preponderance • EEG: characteristic centro-temporal spikes Childhood Absence: • 3 times more common in girls • Triggered by hyperventilation for no less than 3 minutes • Treatment: anti-epileptic drug • Petit mal seizure Febrile Seizure: • A seizure in association with a febrile illness with a temperature greater than 38.4 C (although the fever may not be evident until after the seizure). • Without evidence of any causative disease such as CNS infection, metabolic abnormality, intoxication, etc., in children older than 1 month of age without prior afebrile seizures. Classification: • Simple: o Short o Generalized o Occurs only once in a febrile illness. • Complex: o Prolonged (>15mins) o Focal o Recurrent within a 24-hour period Lumbar Puncture: American Academy of Pediatrics recommendations for Lumbar Puncture: • A must for children under 12 months. • Strongly recommended for children between 12-18 months. • Not necessary in a child > 18 months. • First complex febrile seizures and or persistent lethargy. • Strongly recommended for children who have received prior antibiotic therapy. XI.

PEDIATRIC RHEUMATIC DISEASES:

5 presentations of pediatric rheumatic diseases: • Chronic Arthritis7 • Systemic Connective Tissue Disease • Systemic Vasculitis

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Periodic Fevers Non-articular musculoskeletal pain

Chronic Arthritis (Rheumatic Diseases Presenting as Chronic Arthritis > 6 weeks): • Juvenile Idiopathic Arthritis o Erosive type of arthritis o RF is important for prognosis if JRA will progress to adult RA o Diagnose 6 weeks or more; classify 6 months § Diagnosed as JRA if it lasts > 6 weeks § Onset type defined by type of disease in the first 6 months o ACR criteria for JRA: § Onset: 1 joints § Duration: >6 weeks § Exclusion of other forms of juvenile arthritis o ACR criteria for Classification of JRA: § Polyarthritis - >5 inflamed joints § Oligoarthritis -50% disabling disease (poor prognosis) Arthritis in 1 or more joints plus: Affects 4 or less joints Affects 5 or more joints Often asymmetric Often symmetric • Fever (quotidian pattern) Affects large and small joints • Evanescent rash (rheumatoid Affects large joints (knees/ ankles) rash) • Serositis • Organomegaly • High WBC/ platelets • Elevated acute phase reactants Most difficult to treat Majority achieving remission More prolonged course Poor prognostic factors: Poor prognostic factors for uveitis: Poor prognostic factors: • Polyarticular • (+) ANA • Young age at onset • Fever >3 months • Onset < 6 years • (+) RF/ rheumatoid nodules • Increased inflammatory markers • (+) Anti-CCP >6 months • Large number of affected joints (hips/hands/wrist) **Quotidian fever -1-2 spikes of fever per day with sudden decline to normal or subnormal temperature **Evanescent rash (rheumatoid rash) –salmon pink macular rash, which appears only during fever **Chronic uveitis –can lead to blindness; children usually do not complain of eye pain, photophobia, etc; treat with steroids (MTX) à even arthritis stops; risk factors: oligoarticular, female, young age, (+) ANA

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Juvenile Spondyloarthropathy o Inflammation of the joints of the axial skeleton, large joints of the lower extremities and of entheses (attachment of tendons into the bones) o RF always absent o Types: § Juvenile ankylosing spondylitis § Psoriatic arthirits § Reactive arthritis (Reiter’s syndrome –urethritis, conjunctivitis, arthirits) Arthropathies associated with IBD § Undifferentiated Spondyloarthropathy o High frequency of HLA-B27 o Presence of extra-articular features (eyes, kin, GIT, GUT) o Treatment § Medications • NSAIDS (aspirin, indomethacin, ibuprofen, diclofenac, naproxen) • DMARDS (Sulfasalazine, Methotrexate) • Glucocorticoids • TNF-blockers o Physical and Occupational Therapy o Nutrition

Differentiating Points JRA Younger children F>M Large and small joints Symmetric

JSpA Adolescents M>F Axial and large joint involvement Asymmetric Extra-articular features Associated with HLA-B27

Systemic Connective Tissue Diseases: • Systemic Lupus Erythematosus o Presence of autoantibodies (hallmark of SLE) o Treatment: § Glucocorticoids, Hydroxychloroquine, Immunosuppressive drugs, IV gammaglobulin, biologics 1997 ACR Criteria for SLE (Any 4 of the 11 criteria should be present, serially or simultaneously, during any interval of observation 1. Malar Rash Crosses the nasal bridges Not pathognomonic for SLE 2. Discoid Rash Coin-shaped; affects face and scalp, scars with hyperpigmentation when inflammation resolves 3. Photosensitivity Rashes after exposure 4. Oral or nasopharyngeal ulcers Usually painless; found in palate and nasal mucosa 5. Serositis Pleural and pericarditis effusion 6. Arthritis Non-erosive; intermittent usually in MCPs and PIPs; symmetrical 7. Neurologic diseases Psychosis and/or seizures; (+) anti-ribosomal P and neuronal antibodies 8. Hematologic diseases Hemolytic anemia (Coombs test) Leukopenia (