NUR-507 D4

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Atopic, Rheumatic, and Immunodeficiency Disorders

Chapter 25

Introduction

Atopic disorders, rheumatic disease, immunodeficiencies – shared characteristics

Atopic dermatitis (AD), allergic rhinitis (AR), asthma – triad that may or may not occur

Juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE) – two most common rheumatological diseases in children

Other rheumatological diseases: fibromyalgia, Henoch-Schonlein purpura, Kawasaki disease

Pathophysiology and Defense Mechanisms

Atopic and allergic disorders

Allergens trigger immunoglobulin E (IgE) response

Antigen/antibody union – cascade of biochemical reactions

Four types of allergic reactions

Type I – local and systemic reactions; anaphylaxis

Type II – antibody reactions to antigens on cell surfaces

Type III – deposition of immune micro-precipitates on/around blood vessels

Type IV – delayed hypersensitivity involving T-lymphocytes

Pathophysiology and Defense Mechanisms

Atopic and allergic disorders (Cont.)

Atopy – interaction between genes/environment

Occur only in susceptible individuals

Development of atopy requires

Susceptible person

Exposure to antigen

Predisposition to selective synthesis of IgE

Pathophysiology and Defense Mechanisms

Immune deficiency

Innate immune system of neutrophils, monocytes, macrophages, natural killer cells

Complement system attracts cells to area of inflammation; enhances phagocytosis by opsinins

Adaptive immune system provides more specific responses – lymphocytes (T cells, B cells, natural killer cells)

Pathophysiology and Defense Mechanisms

Rheumatic disorders

JIA, SLE can occur in childhood

Inflammatory changes in connective tissue

Autoimmune basis postulated, but not certain

Periods of remission with SLE

Some children have complete remission of JIA

Laboratory/diagnostic tests – CRP, ESR, ANA, others

Pathophysiology and Defense Mechanisms

General management strategies

Encourage self-care/education

Address burdens of chronic disease

Financial

School, peer, family dynamics

Body image/pain management

Child adjustment/engagement – compliance

Child-parent roles in management

Nutrition/avoidance of obesity

Refer to support groups, resources

Rheumatological Disorders

Juvenile Idiopathic Arthritis

Persistent arthritis >6 weeks/patient <16 years

Likely environmentally induces in genetically predisposed individual

HLA alleles associated with T cell involvement

Infection, trauma may trigger autoimmune reaction

Systemic JIA – uncontrolled activity of innate immune system

Oligoarticular and RF-positive JIA – autoimmunity with involvement of adaptive immune system

Juvenile Idiopathic Arthritis

1 in 1000 children have oligoarticular JIA

Girls > boys in oligoarticular/pauciarticular JIA

Girls = boys in systemic onset JIA

Occurrence and age

Systemic (10%); any age

Polyarticular (40%); late (6-12 years) or early (1-4 years)

Oligoarticular (50%); late or early

Juvenile Idiopathic Arthritis

Clinical findings

History

Pain – mild to moderate aching

Joint stiffness – worse in morning

Joint effusion/warmth

Systemic symptoms: anemia, anorexia, fever, fatigue, lymphadenopathy, salmon-colored rash, weight loss

Juvenile Idiopathic Arthritis

Physical examination

Nonmigratory monoarticular or polyarticular involvement of large or proximal interphalangeal joints more than 3 months

Systemic manifestations

Less common: ocular disease, pleuritis, pericarditis, fatigue, and growth failure

Key physical findings

Joint swelling/effusion

Heat, inflammation of joint

Loss of joint ROM/function; slight flexion/limp

Uveitis may be present

Juvenile Idiopathic Arthritis

Five major types of JIA

Oligoarticular pattern

<4 joints/typically weight-bearing joints in first 6 months

Persistent/extended disease

50% progress to extended disease with involvement of four or more joints after 6 months

Primarily larger joints of knee, wrist, elbow

Systemic symptoms rare

Uveitis in 30%, especially with positive ANA

Juvenile Idiopathic Arthritis

Polyarticular pattern

Five or more joints

RF negative or RF positive

Large or small joint involvement

RF negative/ANA positive similar to extended oligoarticular pattern

RF positive – chronic pain/symmetric joint swelling, low-grade fever, fatigue, nodules

Typically involves small joints of hands, feet, ankles, wrists, knees, cervical spine

Juvenile Idiopathic Arthritis

Systemic JIA

Arthritis in one or more joints for 6 weeks in child <16 years with fever 2 weeks duration with 3 days of daily fever

Fleeting erythematous rash, lymphadenopathy, hepatomegaly, splenomegaly, serositis

Myocarditis/pericardial effusion in 10%

RF rarely positive/ANA positive in 5-10%

10% may develop life-threatening macrophage activation syndrome (MAS)

Juvenile Idiopathic Arthritis

Enthesitis-related JIA

Arthritis of lower limbs – hip, intertarsal joints

Sacroiliac joint involvement later

Inflammation of tendon/ligament insertion, joint capsules

Risk of ankylosing spondylitis in 10-15 years

Occurs in late childhood/adolescence

Juvenile Idiopathic Arthritis

Psoriatic arthritis

More common age 2-4 years and then again age 9-11 years

Family history of psoriasis

Dactylitis or sausage-like swelling of digit

Juvenile Idiopathic Arthritis

Diagnostic studies

Diagnosis of exclusion – based on physical findings

History of arthritis 6 weeks or longer

No diagnostic laboratory test for JIA

Positive RF occurs in <10%

Positive ANA – higher risk of uveitis

Differential diagnosis

Monoarticular arthritis, Lyme disease, toxic synovitis, rheumatic fever, SLE, IBD, septic arthritis, chondromalacia patellae

Juvenile Idiopathic Arthritis

Management

Specialist in pediatric rheumatology

Orthopedists, pain management, cardiologists may be consulted

Therapy depends on local/systemic symptoms

Suppression of inflammation

Preservation of joint function

Prevention of joint deformities

Prevention of blindness

Juvenile Idiopathic Arthritis

Pharmacological therapy

NSAIDs – usually respond well

Ibuprofen: 30-40 mg/kg/day 3-4 divided doses

Tolmetin: 20-30 mg/kg/day; 3-4 divided doses

Naproxen: 10 mg/kg/day; 2 divided doses

Indomethacin – older than 2 years/1-2 mg/kg/day; divided in 2-4 doses; adult/25-50 mg/dose; 2-3 X/day

Celecoxib: (>2 years and adolescents) >10 kg to <25 kg – 50 mg twice daily; >25 kg – 100 mg twice daily

Juvenile Idiopathic Arthritis

Pharmacological therapy (Cont.)

Oral, parenteral, intra-articular corticosteroids

Systemic arthritis – 2 weeks as initial therapy for SIJA with involvement of >4 joints

All other types – prednisone in lowest possible dose with optional intra-articular injection

Juvenile Idiopathic Arthritis

Pharmacological therapy (Cont.)

Disease-modifying anti-rheumatic drugs (DMARDs) – pediatric rheumatologist

Non-biological DMARD

Methotrexate, sulfasalazine, leflunomide

Biological DMARD

Short-acting agents – Anti-IL-1, Anakinra

Long-acting agents – Rilonacept, Cankinumab, Tozilizumab

TNF-α agents – etanercept, infliximab, adalimumab

Juvenile Idiopathic Arthritis

Complications and prognosis

Systemic involvement – iridocyclitis, uveitis, pleuritis, pericarditis, anemia, fatigue, hepatitis

Residual joint damage/granulation tissue

Children with hip involvement, unremitting synovitis, positive RF most likely to have permanent crippling disability

Course of disease variable – no curative treatment

Early aggressive treatment with specialist

Juvenile Idiopathic Arthritis

Patient and parent education and prevention

Risk of Reye syndrome if ASA therapy

Yearly influenza vaccine

Chronic disease counseling/encourage normal play and activities

Side effects of medications

Follow-up with ophthalmologist

Physical therapy mainstay of treatment

Water therapy, hot/cold for pain/stiffness

Low-impact activities

American Arthritis Foundation

IEP at school

Pain management

Systemic Lupus Erythematosus

Chronic, systemic rheumatic disease

Altered immune regulation

Inflammation in multi-organ systems

Blood cells, kidneys, nervous system, skin

Increased circulating autoantibodies

Impaired suppression of autoreactive B-cell clones

Immune complex formation/tissue damage

ANA production/deposition of immune complexes in various tissues

Vasculitis, ischemia, heart, and renal abnormalities

Childhood onset rare; females > males – 4:1 ratio

Systemic Lupus Erythematosus

Clinical findings

Butterfly or malar facial rash

Other findings depend on organ involvement

Abrupt or gradual onset

Fever, rash, fatigue, joint pain typical in children

History

Joint involvement – non-erosive arthritis with tenderness, effusion

Systemic manifestations – fever, rash, proteinuria, seizures, anemia, pleuritis, pericarditis, others

Systemic Lupus Erythematosus

Physical examination

Skin manifestations – pallor, palpable purpura, malar rash, oral ulcerations, gingivitis

Joint tenderness, serositis

Cardiac friction rub

Pleural friction rub

Hepatosplenomegaly/lymphadenopathy

Diagnostic studies

CBC, ANA, ESR, CRP, metabolic screen, UA

Antibody testing

Other tests depending on organ involvement

Systemic Lupus Erythematosus

Differential diagnosis

Infection – bacterial/viral/Lyme

Malignant/autoimmune – leukemia, lymphoma, neuroblastoma

Drug-induced SLE

Management

Referral to rheumatologist

Other subspecialists as needed

Systemic Lupus Erythematosus

Pharmacological management

NSAIDs – for musculoskeletal pain, arthritis

Oral steroids – for renal, cardiac, pulmonary, CNS

Antimalarial drugs – for cutaneous, musculoskeletal manifestations

Immunosuppressant agents – as steroid-sparing agents

Others – monoclonal antibodies

Vitamin D/calcium – to reduce osteoporosis from chronic steroid use

Systemic Lupus Erythematosus

Complications and prognosis

Controllable in children

Severity varies; not always poor prognosis

Renal failure, CNS lupus, myocardial infarction, cardiac failure, infection leading causes of death

UV light exposure may worsen skin lesions and exacerbate systemic problems

Sunscreen, rest

Fibromyalgia Syndrome

Chronic, idiopathic pain syndrome

Diffuse, non-articular musculoskeletal pain and fatigue

Multiple trigger points/discrete painful sites

Onset in adolescents/female>male

Increased pain/sensory feelings in CNS

Fatigue, poor sleep, generalized pain in muscles, ligaments, tendons

Psychosocial stresses, environmental factors, hypersensitivity to stimuli

Fibromyalgia Syndrome

Clinical findings – widespread pain >3 months; tenderness in 11/18 points; waxes/wanes; interferes with ADL

History – pain, fatigue, malaise, paresthesias, insomnia, depression, school absence

Physical examination – pressure causing pain at sites/circumferentially/in linear pattern – commonly at neck, back, lateral epicondyles, greater trochanter, knees. No evidence of arthritis or muscle weakness

Fibromyalgia Syndrome

Diagnostic studies – rule out other disorders

Differential diagnosis – other inflammatory diseases – SLE, other rheumatoid disorders

Management – chronic, lifetime condition

Physical therapy, low-impact aerobics

Muscle strengthening

Psychotherapy/relaxation techniques

Gabapentin to reduce pain sensitivity

Fibromyalgia Syndrome

Patient and parent education

Sleep hygiene

Chronic problem/periods of remission

Prognosis – generally good in children

Chronic Fatigue Syndrome (CFS)

Debilitating/overwhelming fatigue

Idiopathic pain syndromes in children

Persistent, unexplained fatigue of new onset

Not substantially relieved by rest

Reduction in activity

About 2/3 occur after viral pharyngitis/fever

Chronic Fatigue Syndrome (CFS)

Clinical findings

Two necessary findings:

Severe, chronic/debilitating fatigue >6 months

Unexplained by other medical/psychiatric diagnosis

Must have >4 of the following:

Impaired short-term memory/concentration

Recurrent sore throat

Painful cervical or axillary lymph nodes

Myalgia

Headaches of new pattern/severity

Unrefreshing sleep

Postexertional malaise of >24 hours

Chronic Fatigue Syndrome (CFS)

Differential diagnosis – diagnosis of exclusion; rule out hepatitis B or C, SLE, cancer, Lyme disease, alcohol/drug use

Management

Refer to specialist

Pharmacological intervention not effective

Psychological support

Exercise

Majority of pediatric patients will have improvement or complete recovery in 1 to 4 years

Reactive Arthritis Related to Streptococcal Infection

Acute rheumatic fever (ARF)

Post-streptococcal reactive arthritis (PSRA)

Both can occur after group A strep infection

Antistreptococcal immunoglobulins attack human tissues

Strong genetic influence on susceptibility to GAS infection

Evidence of prior GAS infection needed for diagnosis – serology for ASO titers

Post-Streptococcal Reactive Arthritis

During/after GAS infection

Higher incidence in HLA-B27 positive

Persistent joint arthritis

Small/large joints

Axial spine

No cardiac involvement

Resolves without joint damage

Acute Rheumatic Fever

Nonsuppurative complication following Lancefield GAS pharyngeal infection

Autoimmune process involving

Joints – polyarthritis

Heart – rheumatic heart disease

CNS – Sydenham chorea

Subcutaneous tissue – subcutaneous nodules, erythema marginatum

Can recur with subsequent GAS infections

Revised Jones criteria used to diagnose

Acute Rheumatic Fever

Clinical findings and history

Evidence of GAS infection – culture, rapid antigen test, ASO titer

2 major or 1 major/2 minor manifestations

Major manifestations:

Carditis – high-pitched, holosystolic murmur; mitral or aortic regurgitation; eventual mitral valve disease

Polyarthritis – migratory/painful; large joints

Sydenham chorea – uncommon

Erythema marginatum – uncommon

Subcutaneous nodules – uncommon

Acute Rheumatic Fever

Clinical findings and history (Cont.)

Minor manifestations:

Fever

Arthralgia

History of ARF

Diagnostic studies

Elevated acute-phase reactants (ESR, WBC, CRP)

Leukocytosis

Prolonged PR interval on ECG

Acute Rheumatic Fever

Differential diagnosis – associated with rising antibody titers; rule out other diseases of arthritis/arthralgia, connective tissue diseases, IBD, viral and bacterial diseases, Lyme disease

Acute Rheumatic Fever

Management

Antibiotic therapy for GAS – primary prevention to treat within 10 days of onset

If history of ARF, treat URI without positive culture

Anti-inflammatory therapy

ASA – 2 weeks, then tapered

Steroids – severe carditis

Chest radiographs, ECG, echocardiography

Referral for CHF treatment

Bed rest only with CHF

Severe chorea – protect from injury; may use antiepileptic agents

Acute Rheumatic Fever

Prevention of ARF

Treat GAS within 10 days of onset

Antibacterial prophylaxis with prior history

Antibacterial secondary prophylaxis

Penicillin every 4 weeks for 5 years after last episode

Persistent carditis/myocardial or valve disease – treatment may be lifelong

Complications – chronic CHF

Pediatric vasculitis

Henoch-Schonlein Purpura

Most common vasculitis of children

Leukoclastic vasculitis of small vessels

Often preceded by URI

Abdominal pain, palpable petechial/purpural rash on lower extremities, arthritis, renal disease

Cutaneous palpable purpural is hallmark of the disease

Renal manifestations – hematuria, proteinuria, nephritis/nephrotic syndrome; may be chronic

More frequent in winter; males > females

Henoch-Schonlein Purpura

Clinical findings – must include purpura or petechiae in lower extremities with >1 of the following:

Arthritis/arthralgia

Abdominal pain

Histopathology showing immunoglobulin A deposition

Renal involvement with either hematuria or proteinuria

History – previous viral illness; clinical presentation

Henoch-Schonlein Purpura

Physical examination

Rash – pink maculopapular rash; progresses from red to purple to brown palpable purpura

Arthritis

Warmth, swelling, erythema

Periarthritis common in knees/ankles

Diffuse abdominal pain

Edema of scrotum, eyes, hands

Hypertension

Diagnostic studies – clinical findings, UA, BUN, stool guaiac, acute phase reactants

Henoch-Schonlein Purpura

Differential diagnosis – exclude other diseases causing purpura, ITP, post-streptococcal glomerulonephritis, HUS, SLE

Management

Co-manage with pediatric specialists depending on organ system involvement

Hospitalization for moderate-severe GI, renal; or if pulmonary, cardiac, CNS manifestations

Monitor for GI bleeding, hematuria, proteinuria, hypertension

Analgesics and NSAIDs for arthritis

Henoch-Schonlein Purpura

Complications and prognosis

Myositis, orchitis, hemorrhagic cystitis, pancreatitis, cholecystitis, others are infrequent

Arthritis does not recur or cause joint damage

Typically lasts 3-4 weeks/completely resolves

Rash may wax and wane for 1 year

Some children have recurrent disease

Significant nephritis has increased risk for long-term sequelae

Patient and parent education – illness, complications, risk of recurrence; need to monitor for nephritis for 1 year – blood pressure, UA

Kawasaki Disease (KD)

Mucocutaneous lymph node syndrome or infantile polyarteritis

Acute generalized systemic medium vessel vasculitis

Cause unknown – infectious agent may activate immune system in genetically susceptible individual

Geographic/seasonal outbreaks; low person-to-person spread

Kawasaki Disease

EULAR/PREeS classification includes persistent fever for at least 5 days plus four of the following:

Bilateral conjunctival injection

Changes of lips/oral cavity

Cervical lymphadenopathy

Polymorphous exanthema

Changes in peripheral extremities (swell of hands or feet) or perineal area

Kawasaki Disease

Clinical findings

May have atypical/incomplete KD with coronary anomalies on echocardiogram

Atypical KD more common in children <1 year and >9 years of age

Coronary involvement more frequent in incomplete KD

Other clinical features:

Irritability

Aseptic meningitis

Mild acute iridocyclitis/uveitis

Kawasaki Disease

Stage 1: Acute phase – 0 to 14 days

Abrupt onset of high fever unresponsive to antipyretics/antibiotics

Irritability

Bilateral conjunctival injection

Erythema of oropharynx, dryness/fissuring of lips

“Strawberry tongue”

Cervical lymphadenopathy

Polymorphous rash

Urethral erythema

Edema of extremities are typical

Kawasaki Disease

Stage 2: Subacute phase – 2 to 4 weeks

Resolution of fever until all other symptoms resolved

Irritability may be prolonged

Desquamation of fingers, then toes

Transient jaundice, abnormal LFTs

Arthralgia/arthritis

Orchitis

Facial palsy, sensorineural hearing loss

Coronary artery aneurysms appear

Kawasaki Disease

Stage 3: Convalescent phase – 6 to 8 weeks

All clinical signs resolve, but laboratory values not returned to normal

May have nail changes – deep, transverse grooves

Coronary complications may persist into adulthood

Kawasaki Disease

Diagnostic studies

Diagnosis of exclusion

Acute phase reactants may be normal early

WBC may be increased

Thrombocytosis (>1 million) in subacute phase

Stage 1 – elevated ESR, PLT, CRP, leukocytosis

Blood, urine, CSF, GABHS cultures to rule out other sources of fever, depending on symptoms

Echocardiograms at acute illness, 2 weeks, and 6-8 weeks to evaluate coronary, myocardia, pericardial inflammation

Kawasaki Disease

Differential diagnosis

Viral/bacterial infections

Immune-mediated diseases

Mercury poisoning

Tumor necrosis factor receptor associated periodic syndromes

Kawasaki Disease

Management

Early diagnosis to prevent aneurysms

Evoking rapid anti-inflammatory response

Preventing coronary thrombosis by inhibiting platelet aggregation

Minimizing long-term coronary risk factors – lifestyle

IVIG – in first 10 days to reduce incidence of coronary artery abnormalities

High-dose ASA – for anti-inflammatory properties until afebrile for 48-72 hours

Second IVIG dose if IVIG resistant disease

Methylprednisone if IVIG not successful

Kawasaki Disease

Management (Cont.)

Echocardiogram as soon as possible, then at 2 weeks, and 6-8 weeks

Inactivated influenza for all children on chronic ASA; stop ASA if varicella or influenza disease develops to minimize Reye syndrome risk

Delay live-virus vaccines for 11 months after IVIG

Cardiology consult for 1 year in children without coronary changes; indefinitely if coronary changes

Counsel all patients about heart-healthy diet

Kawasaki Disease

Complications and prognosis

Acute disease self-limited

Process of arterial inflammation begins early and may occur as late as 6 months after initial symptoms

Recurrence of KD < 2%

CHF or massive MI, myocarditis, pericarditis 30%

Mortality 1.25% from cardiac complications

Risk of coronary aneurysm reduced if IVIG given within 10 days of onset

Aneurysm regression occurs in half of patients

Atopic disorders

Asthma

Chronic respiratory disease characterized by periods of

Coughing

Wheezing

Respiratory distress

Bronchospasm

Most common chronic respiratory disease of children – up to 35%

Leading cause of emergency department visits

Asthma

Immunohistopathologic responses

Persistent inflammation leads to

Airway remodeling

Acute bronchoconstriction

Airway edema

Mucous plug formation

Hyper-responsiveness to variety of stimuli

Allergens

Cold air

Physical, chemical, pharmacological agents

Asthma

Bronchospasm is often reversible

Wheezing

Breathlessness

Chest tightness

Cough

Remodeling occurs secondary to persistent fibrotic changes starting in childhood – different phenotypes, depending on age of onset

Asthma

Table 25-3 – classification of asthma severity

Depends on symptoms, recurrences, need for medications, pulmonary function measures

Intermittent

Mild persistent

Moderate persistent

Severe persistent

May have mild, moderate, severe exacerbations at any level

Asthma

Early-phase responses (EAR)

Activation of mast cells/mediators

Bronchoconstriction key feature

Starts in 15-20 minutes; resolves in 1 hour after removal of allergen

Late-phase response

Prolonged inflammatory state

Follows EAR within 4-12 hours

Airway hyper-responsiveness more severe

May last from hours to weeks

Asthma

Exercise-induced bronchospasm

Airway narrowing during or minutes after vigorous exercise

May be only trigger for some

Many with asthma have allergic component

Whether hyper-responsiveness present at birth or acquired is unknown

Genetic predisposition to atopy strongest identifiable predisposing risk factor

Asthma

Onset of asthma exacerbations requires

Genetic predisposition

Exposure to environmental factors

Environmental factors

Respiratory virus, mycoplasma, chlamydia

Seasonal patterns

Pollution

Smoking

Psychological stress

Rarely diagnosed before 12 months because of high rates of viral bronchiolitis

Asthma

Increasing incidence of asthma

Prevalence higher in black children

Minority children receive less preventive care

Ambulatory visits

Controller medications

Asthma

Occupational/environmental exposures

Atopic individual response to allergens

Viral, bacterial, mycobacterial infections

Irritants, pollutants, tobacco smoke

GERD

Rapid changes in temperature, barometric pressure

Exercise/psychosocial stressors

Allergic rhinitis/sinusitis

Medications, food additives

Endocrine factors

Asthma

Allergen-induced

House dust mites, cockroaches, indoor molds

Saliva/dander of cats/dogs

Outdoor seasonal molds

Pollens

Food allergy

Asthma

Clinical findings – history

Standardized instruments

Asthma Control Test (ACT), Asthma Control Questionnaire, others

Allows assessment of changes, responses to treatment

Well-controlled children

Symptoms <2 days/week

Use SABA <2 times/week

Poorly controlled

Symptoms >2 days/week

Need step up in medications

Asthma

Clinical findings – history (Cont.)

Family history of asthma/atopy

Conditions associated with asthma – GERD, sinusitis, chronic OM

Chest tightness/dyspnea

Cough/wheezing

Seasonal or continuous symptoms

Recurrent “bronchitis” or pneumonia

Precipitation of symptoms

Asthma

Clinical findings – physical examination

Wheeze, cough

Prolonged expiratory phase

Diminished breath sounds

Increased work of breathing/signs of distress

Vital signs

Cyanosis

Associated findings

Asthma

Diagnostic studies

Pulse oximetry at every assessment

CBC if secondary infection

Routine chest radiographs not recommended

Allergy evaluation

Sweat test

Pulmonary function tests

Peak flow measurements

Exhaled nitrous oxide

Asthma

Differential diagnosis

Acute bronchiolitis, laryngotracheobronchitis

Foreign body aspiration

Congenital cardiac, pulmonary abnormalities

Cystic fibrosis/alpha-1-antitrypsin deficiency

Tracheal compression

Chronic lower respiratory tract infections

Vocal cord dysfunction

GERD

Asthma

Management

Strategies based on severity

Table 25-3 – severity of asthma

Stepwise approach

Assess for compliance and “step up” if needed

If well controlled 3 months, may “step down”

Lowest possible dose of inhaled corticosteroids

Asthma

Chronic asthma

Avoid APAP use

Yearly influenza vaccine

Reduce environmental/allergen exposures

Treat rhinitis, sinusitis, GERD

Consider other pharmacological agents

Anticholinergics

Cromolyn sodium

Leukotriene modifiers

Omalizumab – pulmonologist

Follow-ups with PCP after ED care

Education, including asthma action plan

Asthma

Pharmacological management

Gain control quickly – start at step of initial severity

Systemic corticosteroids may be needed at any time

Inhaled corticosteroids with LABA in some cases

Variations require individualized asthma action plans

MDIs/spacers or nebulizers for β--agonist

Dry powder inhalers do not need spacers

Table 25-7 for comparison of low, medium, high doses of inhaled corticosteroids

Asthma

Acute exacerbations

Based on severity of episode – Table 25-10

Response to initial treatment determines subsequent treatment

Asthma

Drug options in more severe asthma when hospitalized

Magnesium sulfate IV

Ipatroprium oral inhalation

Epinephrine SQ or IM

Heliox to improve drug delivery in airways

Asthma

Complications

Mild secondary respiratory infections

Respiratory distress to arrest

Chronic high-dose steroids – growth retardation

Patient and parent education and prevention

Asthma, control, current level of symptoms

Environmental control

Different medications and how to use

Inhalers, spacers, nebulizers

How to identify symptoms indicating change in therapy/when to seek emergency care

Home PEF or symptom monitoring

Written asthma action plan

Regular follow up

Allergic Rhinitis

Comorbid condition with asthma

Inflammation of nasal epithelium

Antigen-antibody reaction releases chemical mediators

Rhinorrhea, nasal pruritus, sneezing

Second most common atopic disorder

Genetic/environmental factors

Rare under age 6 months

Seasonal, perennial, or episodic

Allergic Rhinitis

Seasonal – hay fever – tree, grass, weed pollen

Perennial – year-round – indoor allergens

Episodic – intermittent exposure to allergen

Clinical findings

Decreased nasal patency/stuffiness

Mouth breathing, snoring, nasal speech

Bogginess of nasal mucosa

Clear, thin, watery to seromucoid rhinorrhea

Nasal crease/“allergic salute”

Itching of palate, pharynx, nose, eyes

Redness of conjunctivae

Enlarged tonsils/adenoids; “cobblestone” pharynx

Allergic Rhinitis

Diagnostic studies/allergy testing

Diagnosis on clinical findings, history of atopy

Nasal eosinophils non-specific finding

Referrals for allergy testing if more severe

Differential diagnosis

Viral URI, sinusitis, FB obstruction, idiopathic rhinitis

Allergic Rhinitis

Management

Avoidance strategies

Determine and remove/avoid triggers

Pharmacological therapy

Oral H1 antihistamines – helpful in seasonal form – second-generation particularly effective

Topical nasal antihistamines – azelastine > 5 years

Decongestants – limited long-term benefits – rebound rhinorrhea

Intranasal steroids – reducing inflammation/obstruction – very effective

Allergic Rhinitis

Management (Cont.)

Allergy immunotherapy and other treatments

Goal to reduce immune tolerance and change response to allergens

Complications and prognosis

Sinusitis, otitis media, malocclusion

Patient and parent education and prevention

Environmental control

Pharmacological therapy

Atopic Dermatitis

Chronic, pruritic, inflammatory skin disorder

Acute/chronic skin eruptions

Facial/extensor pattern in infants/children

The “itch that rashes”

Xerosis, lichenification, cracking

Exact immune mechanism not evident

Epidermal barrier defects/immune dysregulation of immune system, risk of secondary infection

Cytokine production, inflammation, lesions; abnormalities in histamine production

Atopic Dermatitis

Clinical findings

Pruritus/eczematous changes

> 1/3 begin before 3 months – dry skin

Acute manifestations (more common in infants)

Intense itching/redness

Papules, vesicles, edema, serous discharge/crusts

Generalized dry skin

Chronic manifestations (in older children)

Lichenification

Scratch marks

Generalized xerosis

Atopic Dermatitis

Other key features

Tendency for dry skin/itching

Worsens in winter months

Secondary infections

Hyperpigmentation

Diagnostic studies – based on characteristic findings. Immunologic testing not needed

Differential diagnosis – seborrhea, contact dermatitis, nummular dermatitis, psoriasis, scabies

Atopic Dermatitis

Management

Interrupt itch-scratch-itch cycle

Rehydration of stratum corneum

Topical corticosteroids if moisturizing ineffective

Non-soap surfactants

Avoid irritants and allergic triggers

Atopic Dermatitis

Pharmacotherapy

Sedating doses of antihistamines at night

Topical corticosteroids reduce inflammation and pruritus

Seven classes with varying potency

Ointments, gels

Topical calcineurin inhibitors

Prescription emollient devices

Wet wrap therapy

Topical antimicrobials/antiseptics

Atopic Dermatitis

Nonpharmacological therapy

Skin lubrication

Avoidance of triggering factors

Environmental management

Increased environmental humidity

Soaps, detergents, clothing

Allergenic agents

Dietary management

Food allergens not a common trigger

Probiotics not recommended by guidelines

Atopic Dermatitis

Complications and prognosis

Secondary skin infections common

Lichenification associated with chronic itching

2/3 of children will have less severe symptoms; 20% will have complete remission

Patient and parent education and prevention

Recurrent disease that can be controlled

Medications/skin care/environmental controls

Precipitating factors

Pediatric immunodeficiency disorders

Pediatric Immunodeficiency Disorders

Failure of one part of defense system

Results in recurrent infection

Three lines of defense:

External barriers

Innate immunity

Adaptive immunity

Pediatric Immunodeficiency Disorders

Physiology of the immune system

External barriers – physical/mechanical

Skin, epithelium of GI, genitourinary, respiratory tracts

Perspiration, tears, saliva, surfactant, HCl, normal flora

Innate immunity – special cell-pattern recognition

Responds without prior exposure

Activated from direct contact with microbial products

Mediated by neutrophils, macrophages, complement, cytokines

Pediatric Immunodeficiency Disorders

Adaptive immunity – specific recognition

Host responds to specific foreign substance

B and T cells play major role

Antibodies form in response to antigens

100

Pediatric Immunodeficiency Disorders

Primary and secondary immunodeficiency disorders

Primary (PIDD) – usually genetic

Secondary – infections, drugs, renal failure, HIV, leukemia, and lymphomas

Clinical presentation

History of unusual, frequent/recurrent infections

Box 35-4 – 10 warning signs

101

Pediatric Immunodeficiency Disorders

Diagnostic testing

Low lymphocyte count

Complete H&P, CBC with differential, PLT, quantitative immunoglobulins; referral for further testing

Management

Coordinated health services in medical home

Referral to specialists

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Discussion Questions

A 6-year-old has recurrent spring time watery nasal discharge with nasal and eye itch. The common OTC medications have not worked. Discuss the following issues related to this child’s presenting symptoms: (a) the probable diagnosis and its pathophysiology; (b) which if any diagnostic studies should be considered; (c) the likely offending allergens; and (d) key management issues.

A 15-month-old has a recurrent history of erythematous diffuse patches on both cheeks and is unable to sleep at night because of itching. How should this child be managed? Would the management change if a 7-year-old had a similar rash located in the antecubital fossa?

A 10-year-old has had a painful nonerythematous swelling in the right knee for 4 days. The pain is worse in the morning and with exercise. The family denies fever or flulike symptoms. The family history is remarkable for arthritis in the maternal side of the family, and it was noted that they went on a hiking trip in Massachusetts 2 months ago. The rest of history and physical examination is unremarkable. Your suspect JIA but what differential diagnoses should be considered, and which laboratory studies should be ordered?

A 4-year-old is on albuterol MDI prn for rare episodes of wheezing. The family recently moved to an apartment where previous owners had cat. The move has triggered daily wheezing for the past week. Based on NIH guidelines, what medications would be used? What can be done to help her? What is the relationship between allergens and asthma?

103

NUR-507 D4

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