Multi-system disorders Flashcards
What is HSP? Figures?
Henoch-Schönlein Purpura (HSP)
Acute IgA mediated vasculitis of skin, GI, kidneys, joints and sometimes CNS
20/10,000 children per year
More boys than girls
90% present before aged 10
Prodrome of HSP?
Headaches, anorexia and fever
Describe the rash in HSP
Especially involving the legs and buttocks
Erythematous macular/urticarial-> blanching papules-> palpable purpura
What other symptoms/signs are there in HSP?
- Abdominal pain and vomiting (35-85%)
- Joint pain and swelling (60-84%), especially involving the knees and ankles
- Subcutaneous oedema (20-50%)
- Scrotal oedema (2-35%)
- Bloody stools (intussuseption)
- Kidneys: glomerular lesions/capillary proliferation/necrosis/nephrotic syndrome
- Less commonly intracranial haemorrhage/orbital haematomas
How do you diagnose HSP?
By excluding other causes.
• Anti-nuclear antibodies and rheumatoid factor to rule out other autoimmune conditions
• Urinalysis and U&Es for renal function
• D-dimer may be raised
• Immune complexes
• ITP (low platelets)
• Aplastic anaemia (low red, white blood cells and platelets)
Treatment of HSP
- Hydration
- Monitoring for renal complications (eg hypertension, urine dipstick for proteinuria)
- Treating minor symptoms of arthritis, oedema, fever, or malaise
- Discontinuance of any drugs suspected of playing a causative role
- Analgesics: Acetaminophen, Ibuprofen, Naproxen
- Steroids if nephrotic syndrome/severe abdo pain/oedema/neuro involvement
What is kawasaki’s?
Acute febrile vasculitic syndrome of early childhood, occurs after a variety of infections
Can lead to death from coronary artery aneurysm and MI
Symptoms of Kawasaki’s? (11)
- Prolonged fever (5days) +
- Irritability and miserable
- Nonexudative bilateral conjunctivitis (90%)
- Anterior uveitis (70%)
- Perianal erythema (70%)
- Sterile pyuria
- Erythema and oedema on the hands and feet; the latter impedes ambulation
- Strawberry tongue and lip fissures
- Hepatic, renal, and GI dysfunction
- Myocarditis and pericarditis
- Lymphadenopathy (75%); generally, a single, enlarged, nonsuppurative cervical node measuring approximately 1.5 cm
How is Kawasaki’s diagnosed?
- Elevated erythrocyte sedimentation rate (ESR), CRP, Alpha1-antitrypsin, platelets
- Rule out viral and strep/staph infections
- Urine proteins, meprin A and filamin C
Treatment of Kawasaki’s?
- IV Immunglobulins
- Aspirin
- Steroids, infliximab, methotrexate, anticoagulants
What is Lyme’s disease?
Multisystem illness usually caused by infection with the spirochete Borrelia burgdorferi
Transmitted to humans via tick bites, from infected ticks of the genus Ixodes.
Signs and symptoms of Lyme’s
- Erythema migrans (EM) - Rash
- Fever
- Myalgias
- Malaise
- Arthralgia
- Headache
- Tender local adenopathy (local, not diffuse)
- Carditis (heart block)
- Meningism (aseptic meningitis)
- Cranioneuropathy (Bell’s palsy)
What does erythema migrans look like i Lyme’s?
Target lesion
annular homogenous erythema (59%), central erythema (30%), central clearing (9%), or central purpura
Diagnosis of Lyme’s
Clinical (from rash)
1: enzyme immunoassay (EIA) or immunofluorescence assay (IFA) - Total Lyme titer or IgG and IgM titers
2: Western blot testing
LP and ECG needed to investigate meningism/carditis
Treatment of Lyme’s
- Doxycycline, amoxicillin, or cefuroxime axeti if early
- (No Doxy in children)
- If neuro symptoms: IV penicillin, ceftriaxone, or cefotaxime; oral doxycycline
Why is Lyme’s common in Southampton?
New forest deer are a reservoir
What is rheumatic fever?
Acute rheumatic fever (ARF) is an autoimmune inflammatory process that develops as a sequela of group A streptococcal pharyngeal infection. Due to (a) strep toxin (b) abnormal immune response.
Signs and symptoms of rheumatic fever?
- Sore throat in preceding weeks
- Polyarthritis
- Carditis (esp younger children) -> congestive heart failure
- Erythema marginatum
- Chroea (esp in children, usually resolves)
- Subcutaneous nodules (non pus producing)
Is rheumatic fever common?
Hardly ever seen acutely in Western Europe, only as heart disease in the older generation. This decline due to improved socioeconomic conditions & decline in prevalence of the classically described rheumatogenic strains of group A strep
What is JIA?
Juvenile Idiopathic Arthritis
Signs and symptoms of JIA
- Arthritis present for at least 6 weeks before diagnosis (mandatory for diagnosis of JIA)
- Either insidious or abrupt disease onset, often with morning stiffness or gelling phenomenon and arthralgia during the day
- Complaints of joint pain or abnormal joint use
- History of school absences or limited ability to participate in physical education classes
- Spiking fevers occurring once or twice each day at about the same time of day
- Evanescent rash on the trunk and extremities
- Psoriasis or more subtle dermatologic manifestations
Describe the 6 subtypes of JIA
- Systemic-onset juvenile idiopathic arthritis (2 weeks daily fever, rash? Lymph nodes?)
- Oligoarticular juvenile idiopathic arthritis (destructive)
- Polyarticular juvenile idiopathic arthritis (uveitis)
- Psoriatic arthritis (dactylitis/nail pitting/psoriasis in family)
- Enthesitis-related arthritis (inflam at tendon insertion, sacroiliac pain, HLA B27, heel pain)
- Undifferentiated arthritis
Diagnosis of JIA
Diagnosis is by history and examination. Tests could include ANA, RF, ESR, CRP, X-ray, HLA-B27
If only one joint is affected in JIA, what do you need to exclude?
- Soft tissue swelling/joint space narrowing
- Bony erosions/osteopenia/osteoporosis
- Growth disturbances/epiphyseal compression fracture
- Joint subluxation/synovial cysts
- Exclude: osteomyelitis, septic arthritis, trauma, IBD, malignancy, CT disease, sickle cell
