Immunology, Rheumatology, and Allergy

Primary immunodeficiencies

>4 new ear infections

>2 serious sinus infections

>2 pneumonias

>2 months on antibiotics with little improvement

Infant failure to gain weight/grow

Recurrent deep skin and organ abscesses

Persistent thrush and fungal skin infection

IV antibiotics required

Family history

>2 deep seated infections

Primary immunodeficiencies clinical presentations

Syndromic facial features and skeletal anomalies

Ear, chest, skin, and joint infection

Immune organs-tonsils, spleen, lymph nodes

Primary immunodeficiences Management

Post exposure prophylaxis for varicella zoster virus

Avoid live vaccine viruses such as Roto, varicella, MMR, oral polio, and intranasal flu

Give appropriate anabiotic, treatment and prophylaxis

CMV negative blood products for transfusion

Immunoglobin infusion

Will need pneumococcal vaccine

Secondary immuno deficiency

HIV

Prenatal sexual intercourse or contaminated blood

Failure of T cell production

Secondary immune deficiency Presentation

Infant usually asymptomatic can have rash, large lymph nodes, large liver and spleen, cytopenia and growth retardation

Older kids, fever, fatigue, muscle pain, joint pain, weight loss, large lymph nodes, pharyngitis, G.I. upset, peripheral neuropathy

Secondary immuno deficiency, HIV

Must have consent or permission to diagnose—RNA PCR after antibody test testing

Prophylaxis for opportunistic infections

Only vaccinate during high CD4 count

Viral levels monitored every 6 to 12 months

Due to longer survival assist for diabetes, osteoporosis, colon cancer and lipid monitoring

Juvenile idiopathic arthritis

Requires persistence of arthritis for more than six weeks in a child less than 16 years old

JIA subtypes

Oligoarticular-<5 joints

Polyarticular RF negative and positive—-more than 5 joints

Systemic

Psoriatic

JIA presentation

Joint effusion

Tenderness

Restricted range of movement

Systemic= fatigue, weight loss, anemia, fever

Diagnostics JIA

No single test to diagnose JIA

Exam focus on joint swelling, pain, range of motion, flexibility, abnormal gait

ANA is not confirmatory, but does suggest you uveitis

RF factor

JIA management

Occupational and physical therapy

Calcium and vitamin D supplement

NSAIDS

Steroids, DMARDs, and biological

But mainly steroids

SLE Lupus

More in females than males

Malar rash

Discoid lupus rash

Arthritis

Nephritis > proteinuria 0.5gm/day

Encephalopathy-seizures and psychosis

PERICARDITIS AND PLEURITIS

Cytopenia-low rbc,wbc, and platelets

SLE DX

Positive anti-smith, antiphospholipids, and ANA

Requires 4 of 11 criteria over time

CBC- +hemolytic anemia and cytopenia

Elevated ESR

Thyroiditis, autoimmune hepatitis

SLE Management

Steroids*****

DMARDs and other immunosuppressants

Required vaccine-influenza, pneumococcal and meningococcal

Prompt recognition and treatment of infection

Kawasaki second most common vasculitis. Atypical 2 of 5 and Classic 4 of 5

<5 years

Fever for five days

Bilateral painless conjunctivitis without exudate

Strawberry tongue, dry, crack, lips, injected oral mucosa

Polymorphous exanthem

Cervical lymphadenopathy

First redness and swelling of palm and soles followed by peeling of palm soles (convalescent)

Complications of Kawasaki

Heart murmur, valvulitis, myocarditis, pericardial effusion, cardiomegaly, EKG changes, CORONARY ARTERY

Respiratory and pneumonia symptoms

Abdominal pain, nausea/vomiting, diarrhea, and hydros of gallbladder

Arthritis

Dysuria, sterile pyuria, scrotal pain and swelling

Beau lines

Irritability headache and aseptic meningitis

Kawasaki Lab and Radiographic

CBC with leukocytosis-neutrophils

Elevate ESR, CRP, Platelets

Hypoalbuminemia

Anemia

Liver enzymes and GGT

ECHO-decreased ventricular function, dilation, stenosis, aneurysm

ECHO- on diagnosis, 2 weeks, and 6-8 weeks

Kawasaki Management

IVIG -2g/kg

Aspirin 80-100mg/kg/day wean when fever

Once afebrile, antiplatelet dose 3-5mg/kg/day 6-8 weeks

Long term therapy based on risk level

Anaphylaxis

IgE=evil

0.01mg/kg 1:1000

Max for kids 0.3mg

Additional famotidine, albuterol, Benadryl, and METHYLPRED

Fluid bonus