Lupus II and Sjogren's Disease Flashcards
When do you consider autoimmune hemoltyic anemia in a Lupus patient?
- Increased unconjugated bilirubin
- Increased LDH
- Increased reticulocytes
- Reduced haptoglobin
- Positive Coomb’s test
Chronic, low level thrombocytopenia is a characteristic feature of . . .
. . . chronic anti-phospholipid antibody syndrome
Leukopenia occurs in __% of SLE patients.
Thrombocytopenia occurs in __% of SLE patients.
Leukopenia occurs in 50% of SLE patients.
Thrombocytopenia occurs in 25% of SLE patients.
Blood cell and thrombocyte deficiencies may . . .
. . . predate the full emergence of SLE by several years.
When an SLE patient develops leukopenia, what is the differential?
- Leukopenia secondary to chronic immunosuppressive treatment
- Myeloma
- True SLE-induced leukopenia
An SLE patient with renal insufficiency on ACE inhibitors should be monitored closely for ___.
An SLE patient with renal insufficiency on ACE inhibitors should be monitored closely for hyperkalemia.
Autoantibodies appear at a mean time of ___ prior to diagnosis with SLE.
Autoantibodies appear at a mean time of 3.4 years prior to diagnosis with SLE.
Progression of autoantibody development in SLE
Antiphospholipid : t - 3.4 years
Anti-dsDNA : t - 2.2 years
Anti-SM/Anti-RNP : t - 1 year
t = diagnosis with SLE
IF techniques for detecting SLE
- HEp-2 cell substrate technique, add patient’s serum exogenously, very sensitive
- ELISA with patient’s serum against a variety of nuclear antigens, less sensitive but tells you what antibodies you have
- In the clinic: Start w/ ELISA, if negative proceed to HEp-2 assay
- Note that neither of these tests is specific for SLE, ANA may be secondary to several autoimmune conditions or to cancer
- This test is used to rule OUT SLE, not rule it in, its negative predictive value is much greater than its positive predictive value
Anti-dsDNA antibodies correlate well with the presence of ___
Anti-dsDNA antibodies correlate well with the presence of lupus nephritis
If your SLE patient displays low complement levels, you should . . .
. . . test them for a complement deficiency.
While Lupus flares can cause hypocomplementemia through complement consumption, Lupus is also epidemiologically associated with heritable complement deficiencies. So, the complement deficiency may be secondary to the Lupus, or the Lupus may be secondary to the complement deficiency.
Deficiency in the complement components ___ are associated with SLE.
Deficiency in the complement components C4A, C4B, C1q, and C2 are associated with SLE.
___ biopsy may aid in the diagnosis of Lupus.
Histopathologic findings include ___.
Skin biopsy may aid in the diagnosis of Lupus.
Histopathologic findings include basal layer vacuolar degeneration of keratinocytes and interface dermatitis. Immunofluorescence demonstrates deposition of IgG, IgA, IgM, and complement components along the dermoepidermal junction
On skin biopsy, ___ may appear identical to Lupus.
On skin biopsy, dermatomyositis may appear identical to Lupus.
Differential for Lupus
- Viral etiologies
- Parvovirus B19 (sometimes also presenting with ANA and hypocomplementemia)
- Cytomegalovirus
- Epstein-Barr virus
- Acute HIV infection
- Hep B and C (especially with arthritis)
- Malignant etiologies
- Non-Hodgkin’s lymphomas (cytopenias, rash, ANAs)
- Other leukemias or lymphomas
- Other autoimmune diseases
- Dermatomyositis
- Rheumatoid arthritis (arthritis with joint involvement is the same as SLE, but anti-citrullinated antibodies absent in SLE)
- Scleroderma
- Polymyositis
Drug-induced Lupus
- Usually manifests as polyarthritis, myalgia, fever, and serositis
- Minocycline, procainamide, hydralazine, isoniazid, interferon α, and anti-tumor necrosis factor (TNF) agents are well known culprits
- All these drugs cause a positive ANA and, with the exception of minocycline, anti-histone antibodies
Complications of SLE
- Accelerated atherosclerosis
- End-stage renal disease
- Infection (prophylaxis to Pneumocystis jirovecii specifically should be offered)
- Osteoporosis and avascular necrosis (mostly from chronic glucocorticoids, so it is important to use minimum possible dose to control symptoms, but SLE alone is a risk factor for avascular necrosis esp. of femoral head)
- Malignancy (esp. lymphomas (Hodgkin’s and non-Hodgkin’s, lung cancer, cervical cancer)
The highest risk for SLE-induced (or -permitted) cancer is . . .
. . . in the early years after diagnosis, strangely enough. Possibly because pre-cancerous lesions are already present in these patients and are being controlled until SLE or SLE treatment messes with the immune system.
Sjorgen syndrome
a chronic autoimmune disease characterized by lymphocytic infiltration of the salivary, lacrimal, and other exocrine glands, dryness of the eyes and mouth, and circulating autoantibodies
Sjorgen triad
Dry mouth
Dry eyes
Evidence of autoimmune disease
Sjorgen’s syndrome tends to affect. . .
. . . peri-menopausal or postmenopausal women
Sjorgen’s syndrome is associated with a hightened risk for ___.
Sjorgen’s syndrome is associated with a hightened risk for non-Hodgkin’s B cell lymphoma.
Sjorgen salivary gland
Note the chronic inflammation and the arrow depicting a lymphoid follicle and germinal center that has been generated
Healthy minor salivary gland
Hyperlobulation of tongue observed in Sjorgen’s
Bilateral parotid gland swelling
Acutely painful parotid gland swelling usually represents a supervening bacterial parotitis.
Slowly progressive enlargement of the glands should raise concern for the presence of glandular lymphoma, particularly when asymmetric.
Sjorgen’s diagram
Patterns of autoantibody IF staining in SLE and Sjorgen’s
Interpreting the ANA
