Immunology & Rheumatology
- ↑ number of infection
- ≥4otitis media / year
- ≥2 sinus infection or pneumonia/year
- ≥2month AB with little effect
- need for iv AB
- abnormal infections
- ≥2 deep infections/ abscess(skin, organ)
- septicemia
- fungal (persist. oral candida)
- + FH for primary immunodef.
- Primary
- T-Cell disorder
- B-cell disorder (⇒ i.e. IgA deficiency = most common immunodef)
- Phagocyte disorder
- Complement disorder
- Secondary
- Viral (measles, EBV, HIV)
- Metabolic (DM, malnutrition, sickle cell)
- prematurity
- malignancies
- drugs (immunosuppressive)
a. Severe combined immunodeficiency. b. Wiskott-Aldrich syndrome c. Ataxia telangiectasia d. Di George anomaly
a. X-liked agammaglobulinemia b. Common variable immunodeficiency c. Selective IgA deficiency d. AR agammaglobulinemia
a. Chronic granulomatous disease b. Leukocytes adhesion defect c. Chediac-Higashi syndrome d. Myeloperoxidase deficiency e. Cyclic neutropenia
Complement component deficiencies (C1, C2, C3, C4, C5, C6, C7,...)
#1 asymptomatic
- ↑ Infections at sides of mucous membranes→ 🫁Resp + 💩GI + 🍺Urogenital
- Autoimmune disease + atopy, celiac d.
- Patient >4y
- IgA < 10 mg/dL AND IgG+M normal
- Infections → AB
- Vaccination !
Autoimmune disease → chronic synovial inflammation with immune cell infiltration → joint capsule hyperplasia → growth of fibrovascular connective tissue(pannus) → invasion of the articular surface ⇒ loss of joint function
<16y
persists for at least 6 weeks; other known conditions are excluded.
- rheuma-like Arthritis
- swollen, painful movement
- MORNING stiffness + improvement with activity
- Extra-articular “auto-inflammatory” manifestations
- 🔥 fever
- 👁️ uveitis
- 👺 rash
- 🍠HSM + ↑LN
Oligoarticular JIA | Seronegative polyarticular JIA | Seropositive polyarticular JIA | Systemic JIA (Still disease) | Psoriatic JIA | Enthesitis-related JIA | |
Definition | Arthritis involving ≤ 4 joints within 6 months of onset | Arthritis involving more than ≥ 5 joints within 6 months of onset | Arthritis involving more than ≥ 5 joints within 6 months of onset | Arthritis involving ≥ 1 joint AND Intermittent fever lasting for at least two weeks with fever spikes occurring on at least 3 consecutive days AND ≥ 1 extra-articular manifestation | Arthritis and psoriasis OR Arthritis and ≥ 2 of the following features: Dactylitis, Nail changes (pitted nails, onycholysis), Psoriasis first-degree relatives | Arthritis with enthesitis |
Relative Frequency | Most common form (50% of cases) | 30% of cases | < 10% | < 10% | < 10% | 10% |
Pattern of joint involvement | Asymmetrical: Large weight-bearing joints (knee and ankle) usually unaffected. | Symmetrical or asymmetrical: cervical spine and temporomandibular joints also affected. | Symmetrical | Monoarthritis, oligoarthritis, and polyarthritis possible (most commonly affects ≥ 2 joints) | Asymmetrical | Asymmetrical |
Extra-articular manifestations | Chronic anterior uveitis: Seen in ~25% of oligoarticular JIA cases. | Chronic anterior uveitis | - | Transient migratory, macular, salmon-pink rash, Generalized lymphadenopathy, Spleen and liver enlargement, Serositis (peritonitis, pleuritis, pericarditis) | Chronic anterior uveitis, Nail changes, Psoriatic skin lesion often appears after the onset of arthritic symptoms | Acute anterior uveitis anterior: Associated with chronic inflammatory bowel disease, May progress to ankylosing spondylitis |
Laboratory findings | ESR Negative RF, Positive ANA | ESR Negative RF, Positive ANA | ESR Positive RF | ESR, Acute phase reactants Anemia and thrombocytosis, RF negative | ANA positive (in 50% of cases), HLA-B27 may be positive, RF negative | HLA-B27 positive (in 80% of cases), RF negative |
Treatment | NSAIDs, +- intra-articular steroid injections, +- methotrexate | Standard therapy with methotrexate and NSAIDs | Standard therapy with methotrexate and NSAIDs | NSAIDs, Poor response to methotrexate and TNF inhibitors | NSAIDs and intra-articular steroid injections | NSAIDs and intra-articular steroid injections, Sulfasalazine may be used instead of methotrexate |
#1
NSAIDs (Indomethacin)
active arthritis → intra-articular steroids
↓
#2 - controller needed
DMARDs → mainly MTX
↓
#3
Biologicals (i.e. etanercept)
⇒ consider short-term systemic steroids when severe systemic involvement (i.e. serositis) or as bridigung therapy when DMARDs are initiated and control is needed
≥ 4 criteria of MD SOAPBRAIN 📷→ at least 1 clinical + 1 Antibody
anti-phospholipid
check anti-phoslipid Ab + Neuro-workup
- Malar rash
- Discoid rash
- OTHERS ⇒ Lupus pernio, hand rash, livedo reticularis
PREVIOUS URT/Skin GAS INFECTION (2-4w) ⇒ Ab against streptococcal M protein ⇒ molecular mimicry myocardium + nerv ⇒ hypersensitivity reaction against myocardial cells + nerve cells
Major (JONES)
- 🦴Joints: migratory polyarthritis (/ mono)
- 🫀O = Pancarditis (after 2-3weeks)
- 🍨Nodules (subcutaneous - Aschoff) 📷
- ⭕️Erythema marginatum 📷
- 💃Sydenham chorea
Minor
- 🦴joints: athralgia (poly / mono)
- Paraclinic:
- 🫀PR interval prolongation
- 🔥fever (>38,5)
- ♨️acute phase reactants (ESR, CRP, Leukos)
Initial Episode of ARF | Recurrent ARF |
Two major criteria OR One major + two minor criteria | Same as for an initial episode of ARF OR Presence of three minor criteria |
PLUS prev. GAS confirmed(to rule out DDx)
- Drug Tx:
- Penicillin V
- ASS / steroids
- Secondary prevention → AB prophylaxis:
- Ø carditis & Ø residual valvulopathy ⇒ at least until 21y of age OR 5y attack free (whatever is longer)
- + carditis & Ø residual valvulopathy ⇒ at least until 21y OR 10y attack free
- + carditis & + residual valvulopathy
⇒ at least until 40y OR 10y attack free
Tx of first episode of strep throat
(Do rapid strep test in patients with tonsillitis!)
Characteristic | PSRA | Acute RF |
Antecedent Group A | Yes | Yes |
Onset of arthritis after GAS infection | <2 weeks | 2 - 3 weeks |
Migratory Arthritis | No | Yes |
Axial Arthritis | Yes | No |
Heart Involvement | 6% | 50% |
Response to 5-aminosalicylic acid | Not Dramatic | Dramatic |
- NSAID (low response)
- Physical Tx
- AB + prophylaxis (like rheumatic fever)
- APSGN (see above)
Asian <5y
CRASH and BURN on a KAWASAKI
- Conjunvtivits (bilat)
- Rash: Polymorphous non-vesicular
- Adenopathy (cervical)
- Strawberry tongue + cracked red lips
- Hand+foot: red palms + soles
- Burn: Fever >5d
⇒ 📷
CORONARIES!!
→ thrombosis → MI
→ Aneurysm → rupture
→ other 🫀-pathos: Myocarditis, Pericarditis, valvulopathy, arrythmias
F ⇒ most common cause in countries with ↓rheumatic fever incidience
(1) male sex, (2) age <1 or >9 years and, (3) fever> 10 days.
ASS + IVIg
ASS:
⇒ if i.v.Ig fails → Inflixmab, i.v. steroids, plasmapheresis
⇒ if multiple/large coronary aneurysm → anticoagulation
T
T (!)
most common vasculitis in children
FOLLOWING URT infection (1-3w) (A strep)
Iga deposition
IgA:
IgA GN
→ (mainly presenting with isolated) hematuria
→ nephrotic synd
pain + melena (due to inflammation edema + hemorrhage of intestinal submucosa)
→ fecal occult blood test
buttocks + legs
PAPAH: Purpura (palpable)🍓 Abomina Pain 💥 Arthritis 🦴 Hematuria 🥐🩸
Additional:
- Lab:
- IgA
- inflammatory syndrome
- Skin biopsy → IgA depositions
- Urine → hematuria
Non thrombocytopenic palpable purpura + 1 of the following:
- Diffuse, acute, colicky abdominal pain.
- Any biopsy showing predominant IgA deposition (skin, kidney).
- Arthritis (acute, any joint) or arthralgia.
- Renal involvement (any hematuria or proteinuria).
IgA: usually self limited (90%)
⇒ recurrance: #1 NSAIDs → #2 Steroids → #3DMARDS can be given in severe forms of nephritis but usually not needed)
NSAIDS for arthritis → relatively common in IgA
