Rheum Flashcards
IgG4 Disease
1) Bx: Histopath features (4)
2) Common presentations/organs (5)
3) Other organs involved
4) Dx (4 criteria)
1) - Obliterative phlebitis
- Storiform fibrosis
- Lymphoplasmacytic infiltrates (Dense, polyclonal, IgG4-positive Plasma cells per HPF)
- Increased eosinophils
2) - Pancreatitis
- Scloerosing cholangitis
- Sclerosing sialadenitis (aka Mikulicz disease)
- Proptosis
- Retroperitoneal fibrosis (e.g., peri-aortitis, ureteritis with hydronephrosis)
3) Lung, lymphadenopathy, goiter (i.e., Riedel’s thyroiditis), interstitial nephritis, vasculitis, pachymeningitis (dura)
4) - Radiology: Pseudotumor on imaging
- Elevated serum IgG4 level (May also have hypergammaglobulinemia, eosionophilia, & elevated IgE)
- Biopsy result
- Clinical presentation
IgA Vasculitis (Formerly, HSP)
1) Histopath of affected BV (3)
2) Sx Tetrad
3) Epidemiology
1) - Leukocytoclastic vasculitis (neutrophilic infiltration with subsequent perivascular nuclear dust, aka leukocytoclasia)
- BV hemorrhage (RBC extravasation)
- Fibrinoid necrosis (fibrin deposit)
2) - Palpable purpura with lower limb predominance (with NO thrombocytopenia or coagulopathy)
- Arthritis/arthralgis
- Abdominal pain
- Renal diseaser
3) - Most common form of systemic vasculitis in children.
- 90% cases occur in pediatric populations
- Usually self-limiting.
Familial Mediterranean Fever
Dx
RA
1) Scoring
Cspi
Haq
SSx of Vasculitis
1) Large BV
2) Medium BV
3) Small BV
1) Constitutional Sx (Fever, chills, night sweat)), Myalgia, Arthralgia, Vision Change, Jaw (or upper extremity) claudication, asymmetric brachial pluses, and bruits
2) Cutaneous ulcer, abdominal pain, foot drop, wrist drop, and hematuria
3) Palpable purpura, otorrhea, ear pain, muffled sensation in ears, nasal symptoms, sinus pain, wheeze, and hemoptysis.
Ref. BMJ
Systemic Vasculitis
1) Pathology
Sign of immune cell mediated changes in BV wall.
- Fibrinoid necrosis
- Karyorrhexis: Nucleus fragmentation and break up of chromatin into unstructured granules.
- RBC extravasation.
EGPA relapse RF (5)
PR3 ANCA Previous relapse Cardiac involvement Renal involvement
Prednisone
1) MoA
2) SE
3) Monitoring
Ref: Rheuminfo.com
Systemic Vasculitis
2) 2 Broad Types and examples
- Primary (3): Note there is no clear distinction and some diseases can overlap between two sizes.
- Large BV: GCA, Takayasu
- Medium (middle-size) BV: PAN, Kawasaki
- Small BV can be further divided into:
a) Immune complex mediated: IgA vasculitis (HSP), Cryoglobulinemic vasculitis (often associated with HepC), Hypocomplementemic urticarial vasculitis (Anti-C1q), Anti-GBM disease
b) ANCA-associated: MPA, GPA (Wegener’s), eGPA (Churg-Strauss) - Secondary: SLE, Behcet disease, Burerger disease (thromboangiitis obliterans), Sjogren, Neurosarcoidosis, RA, Scleroderma, IBD, Infection (VZV, HepB, HepC), lymphoma, cancer AND Others.
- In general categories, vasculitis due to / or part of: infection, connective tissue disease, other autoimmune condition, iatrogenic (post organ transplantation), CA, etc.
Systemic Vasculitis
3) Mimics
3) Antiophospholipid syndrome, atheroembolic disease, Athermatous vascular disease, cocaine, amphematine, hypersensitivity reaction, infective endocarditis, MM, paraneoplastic, sickle cell, AND secondary causes of vasculitis.
Leukocytoclastic vasculitis
Not a diagnosis. Rather, it is a histopathologic term that decribes microscopic changes with neutrophilic inflammation with fibrinoid necrosis and fragmented neutrophilic nuclei (leukocytoclasis).
- Associated with small vessel vasculitis
Small vessel vasculitis: Characteristics (IgA, cryoglobulins, ANCA, necrotizing granuloma, AND asthma with eosinophilia)
- HSP: IgA dominant immune deposits
- Cryoglobulinemic vasculitis: Has cryoglobulin in blood and vessels.
- MPA: ANCA in blood
- GPA (Wegener’s): ANCA, necrotizing granuloma.
- eGPA (Churg-Strauss): ANCA, necrotizing granuloma, and Asthma with eosinophilia.
Ref: Uptodate
Dermatomyositis
1) Definition
1) Heterogeneous group of muscular disorders characterized by subacute or chronic, progressive muscle weakness.
Dermatomyositis
2) SSx: 5 Key diagnostic features and Many others
2)
- Key features: Difficulty with motor tasks, Muscle weakness, Muscle atrophy, Heliotrope rash with eyelid edema (reddish purple rash on or around the eyelids that is often patchy and uneven), AND Gottron papules (flat-topped, erythematous to violaceous papules and plaques found over bony prominences, particularly MCP, PIP, and/or DIP joints)
- falls, fatigue, weight loss, SOB, abnormal breath sounds, dysphagia, myalgia, arthralgia, palpitation, syncope, rash, calcinosis, joint swelling, arrythmia, peripheral neuropathy
Dermatomyositis
3) Ix: first and Others to consider
3)
- First Ix: CK, EMG, muscle Bx, aldolase, LDH, ALT, and Myoglobin
- Others to consider: ESR, ANA, myositis-specific Ab
- Emerging Ix: CD30, Type 1 IFN, MRI, and US
