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
Dermatomyositis
4) RF
4) - Bimodal age: Children AND age > 40 years
- Exposure to high intensity UV radiation
- Female & Black ethnicity for poly- and dermato-myositis)
- Male & White ethnicity for inclusion body myositis.
- Statin use
- HIV and other infections
Dermatomyositis
4) RF
4) - Bimodal age: around 9 years of age AND 40~70 yo
- Exposure to high intensity UV radiation
- Female & Black ethnicity for poly- and dermato-myositis)
- Male & White ethnicity for inclusion body myositis.
- Statin use
- HIV and other infections
- Family Hx
Dermatomyositis
5) Tx: Acute VS Ongoing (Adult and Child)
5)
- Acute: IV corticosteroid + IVIG
- Ongoing:
a) Adult: Oral corticosteroid + creatine. If not effective, add IVIG. If still not effective, use immunosuppressive agents (instead of IVIG)
b) Child: Pred + MTX.
Dermatomyositis
6) Association with CA
6) 3-6 times increased risk of developing CA: Ovarian CA, pancreatic CA, and NHL
Dermatomyositis
7) Dx
7) - Key SSx (i.e., muscle weakness
- 3 most important Ix: Muscle-derived serum enzyme elevation (CK, LDH, aldolase, myoglobin, and ALT), EMG, and Muscle Bx (for definitive Dx
Dermatomyositis
8) Physical Exam Findings
8) - Heliotrope rash with eyelid edema
- Facial rash
- Gottron papules in MCP, PIP, and/or DIP
- Erythematous rash over knee, elbow, malleoli, and at upper chest (V sign)
- Nail fold changes (dilatation of capillary loops of periungual area)
SLE
1) General info
1) Systemic lupus erythematousus is a Chronic, multisystem disorder that most commonly affects women of childbearing age. Most commonly affects skin and joint. Characterized by presence of ANA.
SLE
2) SSx: Key features (3) AND others
2)
- Key features: Malar rash, photosensitive rash, AND discoid rash
- Other diagnostic features: Arthralgia/arthritis, constitutional Sx, oral/nasal ulcer, alopecia, fibromyalgia, Raynaud, CP, SOB, HTN, signs of nephrosis (edema), lymphadenopathy, thrombosis, Abd pain, n/v/d, limb weakness, dysrrhythmias, dysphagia, CNS abnormality (seizure, psychosis, cognitive deficits, nerve palsies)
SLE
3) RF
3) Female, Age 15~45, African/Asian, drugs, sun exposure, FHx, and Tobacco smoking
