Dyspnea and Cough DSA Flashcards
Granulomatosis with Polyangiitis (Wegener’s granulomatosis) is noted to have a triad of what 3 features?
URT disease
LRT disease
Glomerulonephritis
What is needed for Dx of Polyangiitis with Granulomatosis?
Tissue Bx
ANCAs
Triad
ANCAs of Polyangiitis with Granulomatosis are usually directed against…
Proteinase-3
What is the general pathologic features of Polyangiitis with Granulomatosis? (3)
Vasculitis of small aa., arterioles, and capillaries
Necrotizing granulomatous lesions of URT and LRT
GN
What is the patient demographic of Polyangiitis with Granulomatosis?
4-5th decades, M=F
Annual incidence of 12 per million
How long does it take for Polyangiitis with Granulomatosis to develop? What are some early presentations?
4-12 mo. with fever, malaise, weight loss.
DZs on DDx of Polyangiitis with Granulomatosis
PAN Microscopic polyangiitis Churg-Strauss Chronic sinusitis Goodpasture syndrome SLE Sarcoidosis Cocaine
What 2 lab tests should be ordered for Polyangiitis with Granulomatosis?
c-ANCA
- Abs to proteinase-3 (from neutrophil granules)
- High specificity (>90%)
- If Dz is active, c-ANCA will be high
p-ANCA
- Abs to myeloperoxidase
- Less specific than c-ANCA
- 10-25% w/ classic Polyangiitis with Granulomatosis have p-ANCA if kidney involvement is present
- Proteinuria
- Hematuria and casts
What imaging should be done for Polyangiitis with Granulomatosis?
Chest CT
ANCA is not enough to Dx Polyangiitis with Granulomatosis. What else is paramount?
Bx and histology
- vasculitis
- granulomatous inflammation
- acute/chronic inflammation
- renal Bx for crescents
What is the treatment for Polyangiitis with Granulomatosis is divided into two phases:
How is the therapy determined?
Induction of remission
Maintenance of remission
Determined on whether the pt. has a mild disease (no kidney involvement) vs. severe life/organ-threatening disease (RPGN, Pulm. hemorrhage, etc)
What is the prognosis of Polyangiitis with Granulomatosis without treatment?
What can be given as Tx? Do they work well?
Fatal usually within 1 year.
Cyclophosphamide and Prednisone.
Remissions have been in up to 75%, but half will suffer recurrences.
Churg-Strauss is vasculitis of:
What organ systems are affected?
Small and medium aa. in patients with asthmatic sx.
Skin, lung, maybe heart, GI, nerves.
Major clinical finding of Churg-Strauss:
What is seen on CXR?
*Peripheral eosinophilia
Lung opacities to nodules
What is seen on Bx to make a Dx of Churg-Strauss?
Fibrinoid necrotizing epithelioid and eosinophilic granulomas
What is the Tx for Churg-Strauss initially?
What can it be replaced by for maintenance therapy?
Prednisone and Cyclophosphamide
Cyclophosphamide replaced with Methotrexate or Azathioprine
Populations with increased risk of Sarcoid?
North America AA (F>M)
Northern European white
Onset in 3-4th decade usually
Presenting SX of Sarcoid:
Malaise, fever, DOE Erythema nodosum, iritis, peripheral neuropathy, arthritis, CM may prompt initial eval. Can be ASX. Parotid gland enlargement HSM, Lymphadenopathy
Myocardial sarcoid is found in only 5% of patients, but it can lead to: (3)
Restrictive CM
Cardiac dysrhythmias
Conduction disturbances
Lab abnormalities in Sarcoid
Leukopenia
Possible hypercalcemia
Elevated ACE
Sarcoid on PFT
Restrictive changes with decreased lung volumes and diffusion capacity most common. May reveal airflow obstruction.
What test is positive in 70% of patients with Sarcoid?
Skin test anergy
Stage I, II, III and IV Sarcoid on imaging
Stage I - hilar adenopathy
Stage II - hilar adenopathy w/ parenchymal involvement
Stage III - only parenchymal involvement
Stage IV - advanced fibrotic changes in upper lobes
What is useful for Dx of Sarcoid?
Bx of non-caseating granulomata
Transbronchial Bx
ECG may show conduction problems and dysrhythmias
What is often seen in bronchoalveolar lavage in Sarcoid?
What is it useful for?
Increased lymphocytes with a high CD4/CD8 ration
Used to follow Dz, not for Dx
What is given for Tx of Sarcoid?
What immunosuppressives are given?
Prednisone
Methotrexate or Cyclosporine, or anti-TNF w/ Infliximab
Demographic of patients with IPF
6-7th decade, M>F
More common in past smokers
What genetic variants (mutations) are associated with IPF? (3)
Mutations in surfactant protein C
Telomerase
Polymorphisms of MUC5B
Presentation of IPF
**Worsening DOE Dry cough Basilar crackles Clubbing Cyanosis RSHF
Workup for IPF
CT/CXR PFT 6 min walk Labs **Lung Bx
Meds for IPF (2) and their S/E
Pirfenidone - anti-fibrotic med. No known MOA. Taken 3x/day. S/E are nausea, abdominal pain, photosensitivity. Must monitor LFTs.
Nintedanib - tyr kinase inhibitor. S/E are diarrhea. LFTs must be monitored.
Aside from the meds, what can be given to patients with IPF?
O2
Pulm rehab
Transplant
Risk factors for PE
Immobility Hyperviscosity Increased venous pressures Vessel damage Hypercoagulative states
