Vasculitis
Complement activation through this pathway is important in ANCA associated vasculitis pathogenesis
What is the alternative pathway?
These are the 3 most common organisms to cause pyogenic sacroiliitis
What are Staph aureus, Group B strep, and Pseudomonas
The autoinflammatory syndrome characterized by bone marrow biopsies with cytoplasmic vacuolization, hypercellularity, granulocytic hyperplasia, and an increased myeloid-to-erythroid ratio is caused by a mutation in this gene or enzyme
UBA1 (ubiquitin)
This is the timeframe for OP treatment initiation after a fragility fracture
What is 2-12 weeks?
Name a first-line contraceptive for a patient with high SLE disease activity?
Progestin-only pill or IUD
This is the best imaging modality is best to assess for pulmonary artery aneurysms in Behcet's
What is a Spiral CTA of chest?
not MRA of chest, because it lacks sensitivity to detect medium and small vessels, and venulitis. Also cannot comment on lung parenchyma.
This is the most likely infectious agent in an anemic immunosuppressed lupus patient; bone marrow bx shows pure red cell aplasia with giant proerythroblasts.
What is Parvovirus B19?
This childhood periodic fever syndrome can be treated with tonsillectomy
Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA)
This is the drug of choice for GIOP prevention in a moderate-risk PREGNANT patient
What is risedronate?
A mutation in this gene may cause early chondrocalcinosis and pseudogout
What is ANKH (ankylosis protein homolog)?
This is the typical description of behcet's ocular involvement (which area(s) involved)
What is panuveitis?
These are the recombinant zoster vaccination recommendations in an immunosuppressed patient?
Age, Number, Interval
19 years old or more
2 doses
1-2 months apart
These three CAPS diseases (cryopyrin-associated periodic fever syndromes) are associated with NLRP3 gain-of-function mutations
What are
FCAS (Familial Cold autoinflammatory syndrome)
MWS (Muckle-Wells Syndrome)
CINA/NOMID (Chronic Infantile Neurologic Cutaneous and Arthritis Syndrome)
This is the diagnosis and treatment of a patient with loss of adult dentition, calcific periarthritis, recurrent metatarsal stress fractures
What is asfotase alfa (enzyme replacement) for hypophosphatasia?
mutation of tissue-nonspecific alkaline phosphatase (TNSAP)
This mediator of Anemia of Chronic Disease inhibits duodenal iron absorption
What is hepcidin?
This is the most likely diagnosis in a patient with a "good GCA history" and temporal artery biopsy showing
-panarteritis with fragmentation of the internal elastic lamina
-rich inflammatory infiltrate of macrophages and CD4+ T cells associated with bands of fibrinoid necrosis
What is GPA?
This is the most likely infectious agent in a farmworker who develops cryoglobulinemic vasculitis
What is Coxiella burnetii (Q fever)?
This is the diagnosis and treatment for a 2-year-old with livedo, recurrent strokes, and polyarthritis
What is TNFi therapy for DADA2?
This is the preferred form of calcium supplementation in a patient taking PPI or H2i
What is calcium citrate?
Calcium carbonate acid for absorption and is better absorbed with meals
This is the treatment for an elderly woman presenting for medial knee pain, most prominent over medial condyle, found to have a 3cm area of osteonecrosis
What is monitoring for regression?
Spontaneous osteonecrosis of the knee
TX: depends on size of lesion
-if less than 3.5cm, usually regress
-if more than 3.5cm, surgery
This is the classic finding in a HSP biopsy (be as specific as possible)
What is vasculitis of postcapillary venules?
This hereditary periodic fever syndrome is protective against the bubonic plague
What is FMF?
A mutation in this gene may cause Pyogenic Arthritis, Pyoderma gangrenosum, Acne syndrome
What is PSTPIP1?
Proline-Serine-Threonine Phosphatase Interacting Protein 1, is a protein involved in various cellular functions, including T-cell activation, cell migration, and interleukin release
This enzyme, which normally promotes promoting attachment of the osteoclast to the bone, is inhibited by an anti-osteoporotic treatment
farnesyl pyrophosphate synthase (inhibited by bisphosphonates)
This is the most likely type of amyloidosis in a patient on hemodialysis
What is beta-2 microglobulin?