Autoimmune systemic disroders in Rheumatology (week 11) Flashcards
———— : immunoglobulin A vasculitis (IgAV), is the most common systemic vasculitis of childhood
Henoch-Schönlein purpura (HSP)
CF of Henoch-Schönlein purpura (HSP)
characterized by a tetrad of CM:
A. Palpable purpura without thrombocytopenia and coagulopathy
B. Arthralgia and/or arthritis
C. Abdominal pain
D. Renal disease
———- : disease affecting medium sized vessles, also called mucocutaneous lymph node syndrome is one of the most common vasculitides of
childhood
Kawasaki disease
* Autoimmune systemic disorder
CF of Kawasaki disease
characterized by:
1) systemic inflammation manifested by fever and mucocutaneous involvement,
2) bilateral nonexudative conjunctivitis (characteristic for KD),
3) erythema of the lips and oral mucosa,
4) rash,
5) extremity changes (peeling of the skin),
6) cervical lymphadenopathy
* these symptoms are normally not present at the same time
Diagnosis criteria for Kawasaki disease
fever for ≥5 days,including at least 4 of other signs of mucocutaneous inflammation:
1) changes of extremities
2) polymorphous exanthema
3) bilateral conjuctivitis
4) rash
5) lyphadenopathy
———- : uncommon chronic vasculitis of unknown etiology, which primarily affects the aorta and its primary branches.
* Affects W>M
Takaysau arteritis (TAK)
In TAK disease The inflammation may be localized to a portion of the ——- or ———- and ——-, or may involve the entire vessel
In TAK disease The inflammation may be localized to a portion of the thoracic or abdominal aorta and branches, or may involve the entire vessel
Symptoms of Takaysau arteritis
1) cyanosis and/or
2) pain in arms or legs (limb claudication),
2) lightheadedness
- normally its an asymptomatic disease that is diganosed based on measurements of Bp, palpation of pulses, identification of Bruits, and careful cardiac auscultation
The physical examination of a patients with TAK should particularly focus on accurate measurements of ——-**, ———-, identification of ——–, and careful —————–
The physical examination of a patients with TAK should particularly focus on accurate measurements of bp, palpation of pulses (irregualr/ absent), identification of bruits, and careful cardiac auscultation
CM/Diagnosis of Behçet’s disease
Recurrent oral aphthae (at least three times in one year) plus two of the following:
- Recurrent genital aphthae
- Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit lamp examination, or retinal vasculitis)
- Skin lesions (including erythema nodosum, pseudo-vasculitis, papulopustular lesions, or acneiform nodules consistent with Behçet’s)
- A positive pathergy test
* Pathergy : skin lesions due hyper-reactivity to minimal trauma
Diagnosis of Behçet’s disease
———– (at least three times in one year) plus two of the following:
- ————–
- Eye lesions (including anterior or posterior ——, cells in vitreous on slit lamp examination, or ———)
- Skin lesions (including ——–, ——–, ———–, or acneiform nodules consistent with Behçet’s)
- A positive ———- test
Recurrent oral apathae (at least three times in one year) plus two of the following:
- Recurrent genital apathae
- Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit lamp examination, or retinal vasculitis)
- Skin lesions (including erythema nodosum, pseudo-vasculitis, paplopustular lesions, or acneiform nodules consistent with Behçet’s)
- A positive pathergy test
c-ANCA ?
Wegener granulomatosi
p-ANCA
1) Microscopic polyangiitis;
2) Churg-Strauss syndrome
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome) is a multisystem disorder chracterisized by?
1) chronic rhinosinusitis
2) asthma (cardinal sign)
3) prominent peripheral blood eosinophilia (≥ 1500 cells/microL and/or >10 percent eosinophils on differential leukocyte count)
Cardinal feature on Chrug-Strauss Syndrome
Asthma
what do you observe? which disease is this?
Visibly enlarged temporal artery in a patient with giant cell arteritis
