CHAPTER 29-SYSTEMIC LUPUS ERYTHEMATOSUS Flashcards
1
Q
What are the different forms of lupus
A
Discoid (cutaneous) lupus; systemic lupus; drug-induced lupus; neonatal lupus
2
Q
What is discoid lupus
A
Form of lupus limited to the skin
3
Q
Does discoid lupus generally involve internal organs
A
No
4
Q
What percentage of lupus cases are discoid lupus
A
Approximately 10%
5
Q
What is systemic lupus
A
Systemic form that can affect skin
6
Q
What percentage of lupus cases are systemic lupus
A
Approximately 70%
7
Q
In systemic lupus
A
what percentage of cases involve a major organ
8
Q
What is drug-induced lupus
A
Lupus-like syndrome caused by certain prescribed drugs
9
Q
What are the two most common drugs causing drug-induced lupus
A
Hydralazine hydrochloride; procainamide hydrochloride
10
Q
What are clinical features of drug-induced lupus
A
Similar to systemic lupus but milder
11
Q
What happens to symptoms of drug-induced lupus after discontinuation of the drug
A
Symptoms usually fade
12
Q
What is neonatal lupus
A
Rare condition acquired from maternal autoantibodies (anti-Ro/SS-A or anti-La/SS-B) affecting fetus/newborn
13
Q
What are common features of neonatal lupus
A
Rash within first weeks of life (may persist up to 6 months); congenital heart block is less common
14
Q
Is neonatal lupus the same as systemic lupus
A
No
15
Q
What is the cause of systemic lupus erythematosus (SLE)
A
Idiopathic; primary defect in immune regulation is important
16
Q
What factors may trigger SLE
A
Genetic predisposition; hormones; environmental factors (infections
17
Q
What is the role of hormonal influences in SLE
A
More common in women
18
Q
How does pregnancy affect lupus
A
Symptoms may worsen during pregnancy and postpartum; increased risk of complications
19
Q
What is antiphospholipid syndrome
A
Condition secondary to lupus with antibodies against coagulation factors
20
Q
What is the risk for children of lupus patients developing lupus
A
About 5%
21
Q
What is the risk for siblings or parents of lupus patients developing lupus
A
About 10%
22
Q
What environmental factors can induce or exacerbate SLE
A
UV light; bacterial and viral infections
23
Q
How does UV light contribute to SLE
A
May cause DNA changes (thymine dimers) increasing antigenicity and anti-DNA formation
24
Q
What is the estimated incidence of SLE
A
50 to 70 new cases per year per 1 million population
25
What is the prevalence of SLE in women aged 20–64
1 in 700
26
What is the overall prevalence of SLE
1 in 2000
27
Which racial groups have increased frequency of SLE
Blacks
28
What is the estimated 10-year survival rate for SLE
Greater than 90%
29
What are the two most frequent causes of death in SLE
Renal failure; infectious complications
30
What are some drugs that can produce clinical and serologic features of SLE
Procainamide hydrochloride (antiarrhythmic)
31
What is one of the most common clinical manifestations of SLE
Fever
32
What elevated antibody titers are characteristic of lupus disease activity
Anti-dsDNA; anti-ribosomal P antibodies
33
What serologic findings are characteristic of lupus disease activity
Reduced levels of complement; leukopenia
34
What mediates tissue damage from vasculopathy in SLE
Immune complexes
35
What are some direct effects of antibodies to cell surface molecules or serum components in SLE
Thrombocytopenia; antiphospholipid syndrome
36
What are the range of manifestations in SLE
From mild photosensitive rash and arthritis to life-threatening involvement of CNS
37
What are the most common clinical manifestations of SLE
Polyarthritis; dermatitis
38
What is the typical course of SLE
Chronic and irregular
39
What are common clinical signs and symptoms of SLE
Fever; weight loss; malaise; arthralgia and arthritis; erythematous maculopapular rash over the bridge of the nose
40
What is a common tendency in SLE
Increased susceptibility to common and opportunistic infections
41
What can trigger the onset of lupus
Sun exposure; infections; pregnancy or after delivery
42
What skin-related sign is often observed in SLE patients
Alopecia (hair loss)
43
What is photosensitivity in the context of SLE
Unusual skin reaction to sunlight
44
What are discoid lesions
Circular raised patches on the skin
45
What renal characteristics can be observed in SLE patients
Focal or diffuse glomerulonephritis
46
What is the prevalence of clinically significant kidney disease in SLE patients
Occurs in about 50% of SLE patients
47
What are the clinical manifestations of lupus nephritis
Proteinuria; hematuria; cellular casts; renal insufficiency
48
What hematologic findings are common in SLE
Anemia; leukopenia; thrombocytopenia
49
What is lymphadenopathy
Enlargement of lymph nodes
50
What is serositis
Inflammation of serous membranes (pleura
51
What cardiopulmonary characteristics are associated with SLE
Pericarditis; pleuritis; myocarditis; endocarditis
52
What gastrointestinal manifestations can occur in SLE
Abdominal pain; nausea; vomiting; pancreatitis
53
What musculoskeletal features are typical of SLE
Arthralgia; arthritis (often non-erosive); myalgia
54
What neuropsychiatric features can be observed in SLE patients
Seizures; psychosis; cognitive dysfunction
55
What is late-onset lupus
Diagnosis of SLE after age 50
56
What cellular abnormalities are observed in SLE,
Defects in T cells; B cells; antigen-presenting cells
57
What T cell abnormalities are seen in SLE,
Reduced numbers; impaired function; increased apoptosis
58
What B cell abnormalities are observed in SLE,
Hyperactivity; increased autoantibody production
59
What is the role of antigen-presenting cells in SLE,
Abnormal antigen presentation can trigger autoreactive T cells
60
What humoral abnormalities are characteristic of SLE,
Autoantibody production; complement abnormalities
61
What autoantibodies are commonly found in SLE,
Anti-nuclear antibodies (ANA); anti-dsDNA; anti-Sm; anti-Ro/SSA; anti-La/SSB; antiphospholipid antibodies
62
What complement abnormalities are observed in SLE,
Decreased levels of complement components (C3, C4, CH50)
63
What are the immunologic consequences of SLE,
Tissue damage; inflammation; immune complex deposition
64
What mechanisms mediate tissue damage in SLE,
Autoantibodies; immune complexes; complement activation; cellular cytotoxicity
65
Where do immune complexes typically deposit in SLE,
Kidneys; skin; joints; blood vessels
66
What histologic changes are seen in affected organs in SLE,
Inflammation; vasculitis; fibrosis; immune complex deposition
67
What are the typical hematologic findings in SLE,
Anemia; leukopenia; thrombocytopenia
68
What hemostatic abnormalities are associated with SLE,
Antiphospholipid syndrome; lupus anticoagulant
69
What are the typical serologic findings in SLE,
Positive ANA; elevated levels of specific autoantibodies; decreased complement levels
70
What autoantibodies are highly specific for SLE,
Anti-dsDNA; anti-Sm
71
What is the significance of antiphospholipid antibodies in SLE,
Associated with thrombosis and pregnancy complications
72
What ANA patterns are commonly observed in SLE,
Homogeneous; speckled; rim (peripheral)
73
What is the clinical significance of a positive ANA test,
Suggests possible autoimmune disease, particularly SLE
74
How are specific autoantibodies identified after a positive ANA test,
Using ENA (extractable nuclear antigen) panel
75
What is the role of complement testing in SLE diagnosis,
Helps assess disease activity and severity
76
What is one of the most common clinical manifestations of SLE
Fever
77
What elevated antibody titers are characteristic of lupus disease activity
Anti-dsDNA; anti-ribosomal P antibodies
78
What serologic findings are characteristic of lupus disease activity
Reduced levels of complement; leukopenia
79
What mediates tissue damage from vasculopathy in SLE
Immune complexes
80
What are some direct effects of antibodies to cell surface molecules or serum components in SLE
Thrombocytopenia; antiphospholipid syndrome
81
What are the range of manifestations in SLE
From mild photosensitive rash and arthritis to life-threatening involvement of CNS
82
What are the most common clinical manifestations of SLE
Polyarthritis; dermatitis
83
What is the typical course of SLE
Chronic and irregular
84
What are common clinical signs and symptoms of SLE
Fever; weight loss; malaise; arthralgia and arthritis; erythematous maculopapular rash over the bridge of the nose
85
What is a common tendency in SLE
Increased susceptibility to common and opportunistic infections
86
What can trigger the onset of lupus
Sun exposure; infections; pregnancy or after delivery
87
What skin-related sign is often observed in SLE patients
Alopecia (hair loss)
88
What is photosensitivity in the context of SLE
Unusual skin reaction to sunlight
89
What are discoid lesions
Circular raised patches on the skin
90
What renal characteristics can be observed in SLE patients
Focal or diffuse glomerulonephritis
91
What is the prevalence of clinically significant kidney disease in SLE patients
Occurs in about 50% of SLE patients
92
What are the clinical manifestations of lupus nephritis
Proteinuria; hematuria; cellular casts; renal insufficiency
93
What hematologic findings are common in SLE
Anemia; leukopenia; thrombocytopenia
94
What is lymphadenopathy
Enlargement of lymph nodes
95
What is serositis
Inflammation of serous membranes (pleura
96
What cardiopulmonary characteristics are associated with SLE
Pericarditis; pleuritis; myocarditis; endocarditis
97
What gastrointestinal manifestations can occur in SLE
Abdominal pain; nausea; vomiting; pancreatitis
98
What musculoskeletal features are typical of SLE
Arthralgia; arthritis (often non-erosive); myalgia
99
What neuropsychiatric features can be observed in SLE patients
Seizures; psychosis; cognitive dysfunction
100
What is late-onset lupus
Diagnosis of SLE after age 50
101
What cellular abnormalities are observed in SLE
Defects in T cells; B cells; antigen-presenting cells
102
What T cell abnormalities are seen in SLE
Reduced numbers; impaired function; increased apoptosis
103
What B cell abnormalities are observed in SLE
Hyperactivity; increased autoantibody production
104
What is the role of antigen-presenting cells in SLE
Abnormal antigen presentation can trigger autoreactive T cells
105
What humoral abnormalities are characteristic of SLE
Autoantibody production; complement abnormalities
106
What autoantibodies are commonly found in SLE
Anti-nuclear antibodies (ANA); anti-dsDNA; anti-Sm; anti-Ro/SSA; anti-La/SSB; antiphospholipid antibodies
107
What complement abnormalities are observed in SLE
Decreased levels of complement components (C3
108
What are the immunologic consequences of SLE
Tissue damage; inflammation; immune complex deposition
109
What mechanisms mediate tissue damage in SLE
Autoantibodies; immune complexes; complement activation; cellular cytotoxicity
110
Where do immune complexes typically deposit in SLE
Kidneys; skin; joints; blood vessels
111
What histologic changes are seen in affected organs in SLE
Inflammation; vasculitis; fibrosis; immune complex deposition
112
What are the typical hematologic findings in SLE
Anemia; leukopenia; thrombocytopenia
113
What hemostatic abnormalities are associated with SLE
Antiphospholipid syndrome; lupus anticoagulant
114
What are the typical serologic findings in SLE
Positive ANA; elevated levels of specific autoantibodies; decreased complement levels
115
What autoantibodies are highly specific for SLE
Anti-dsDNA; anti-Sm
116
What is the significance of antiphospholipid antibodies in SLE
Associated with thrombosis and pregnancy complications
117
What ANA patterns are commonly observed in SLE
Homogeneous; speckled; rim (peripheral)
118
What is the clinical significance of a positive ANA test
Suggests possible autoimmune disease
119
How are specific autoantibodies identified after a positive ANA test
Using ENA (extractable nuclear antigen) panel
120
What is the role of complement testing in SLE diagnosis
Helps assess disease activity and severity
121
What is the purpose of the rapid slide test for antinucleoprotein
To detect the presence of antinuclear antibodies (ANA) in patient serum
122
What type of specimen is required for the rapid slide test
Patient serum or plasma
123
What is the principle behind the rapid slide test
Patient serum is mixed with reagent cells containing nuclear antigens; agglutination indicates presence of ANA
124
What is the appearance of a positive test result in the rapid slide test
Visible clumping or agglutination on the slide
125
What is the importance of using reagent cells in the rapid slide test
They provide a standardized source of nuclear antigens for antibody binding
126
What is the general procedure for performing the rapid slide test
Mix equal volumes of patient serum and reagent cells on a slide; rotate gently; observe for agglutination within minutes
127
What are common causes of false positive results in the rapid slide test
Rheumatoid factor; nonspecific agglutinins; improper technique
128
How should a positive rapid slide test be confirmed
By performing indirect immunofluorescence assay (IFA) for ANA and further specific antibody testing
129
What is the clinical significance of a positive rapid slide test
Indicates possible autoimmune disease requiring further evaluation
130
What precautions should be taken when performing the rapid slide test
Use proper controls; avoid contamination; follow timing strictly
131
What is the fundamental cause of autoimmune diseases
Loss of immunologic tolerance to self-antigens
132
What are the three main categories of autoimmune disorders
Organ-specific; midspectrum; organ-nonspecific
133
What role do autoantibodies play in autoimmune diseases
They can cause tissue damage directly
134
What genetic factors influence susceptibility to autoimmune diseases
HLA haplotypes and other immune-related genes
135
How do environmental factors contribute to autoimmunity
By triggering immune responses through infections
136
What is the difference between central and peripheral tolerance
Central tolerance eliminates autoreactive lymphocytes during development; peripheral tolerance controls autoreactive cells in the periphery
137
What are common laboratory tests used to diagnose autoimmune diseases
ANA test
138
How does the direct antiglobulin test help in diagnosing autoimmune hemolytic anemia
By detecting antibodies or complement bound to red blood cells
139
What is the clinical importance of anti-dsDNA antibodies in SLE
They correlate with disease activity and renal involvement
140
How does immunosuppressive therapy help in autoimmune diseases
By reducing immune system activity to prevent tissue damage
141
What are potential side effects of immunosuppressive therapy
Increased risk of infections
142
What is the purpose of allergen immunotherapy
To induce immune tolerance and reduce allergic symptoms
143
How do monoclonal antibodies like rituximab work in autoimmune diseases
By targeting and depleting B cells that produce autoantibodies
144
What is the significance of molecular mimicry in autoimmunity
Pathogens share epitopes with self-antigens
145
What is the role of regulatory T cells in preventing autoimmunity
They suppress autoreactive immune responses and maintain tolerance
146
What is the definition of malar rash in SLE
Fixed erythema
147
What are the characteristics of discoid rash in SLE
Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions
148
How is photosensitivity defined in the context of SLE
Skin rash as a result of unusual reaction to sunlight by patient history or physician observation
149
What are the characteristics of oral ulcers in SLE
Oral or nasopharyngeal ulceration
150
What are the features of nonerosive arthritis in SLE
Involving two or more peripheral joints
151
What are the features of pleuritis or pericarditis in SLE
Pleuritis—convincing history of pleuritic pain or rubbing heard by a physician
152
What are the features of renal disorder in SLE
Persistent proteinuria >0.5 g/day
153
What are the features of neurologic disorder in SLE
Seizures—in the absence of offending drugs or known metabolic derangements (e.g.
154
What are the features of hematologic disorder in SLE
Hemolytic anemia—with reticulocytosis OR Leukopenia—<4000/mm3 total on two or more occasions OR Lymphopenia—<1500/mm3 on two or more occasions OR Thrombocytopenia—<100
155
What are the immunologic disorder criteria in SLE
Anti-DNA—antibody to native DNA in abnormal titer OR Anti-Sm—presence of antibody to Sm nuclear antigen OR Positive finding of antiphospholipid antibodies on: An abnormal serum level of IgG or IgM anticardiolipin antibodies
156
What is the definition of positive antinuclear antibody
An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with drug-induced lupus syndrome
157
What is the diagnostic criteria for SLE according to the American College of Rheumatology
Presence of 4 or more of the 11 criteria
158
What is the importance of the ANA test in SLE diagnosis
ANA is sensitive but not specific; it is used as a screening test for SLE and other autoimmune diseases
159
What are the limitations of the ANA test
Positive ANA can be found in other autoimmune diseases
160
What is the significance of anti-dsDNA antibodies in SLE
Highly specific for SLE and correlate with disease activity and renal involvement
161
What is the clinical relevance of anti-Sm antibodies
Highly specific for SLE but less sensitive; presence confirms diagnosis
162
What is the role of antiphospholipid antibodies in lupus
Associated with increased risk of thrombosis
163
What laboratory methods are used to detect ANA
Indirect immunofluorescence assay (IFA) on Hep-2 cells is the gold standard
164
What are common ANA staining patterns
Homogeneous
165
What is the clinical significance of the homogeneous ANA pattern
Associated with SLE and drug-induced lupus
166
What is the clinical significance of the speckled ANA pattern
Seen in SLE
167
What is the clinical significance of the nucleolar ANA pattern
Associated with systemic sclerosis
168
What is the clinical significance of the centromere ANA pattern
Associated with limited systemic sclerosis (CREST syndrome)
169
What is the principle of the ENA panel
Detection of autoantibodies to extractable nuclear antigens for more specific diagnosis
170
What autoantibodies are commonly included in the ENA panel
Anti-Ro (SSA)
171
What is the clinical significance of anti-Ro (SSA) and anti-La (SSB) antibodies
Associated with Sjögren’s syndrome and neonatal lupus
172
What is the clinical significance of anti-Scl-70 antibody
Associated with diffuse systemic sclerosis
173
What is the clinical significance of anti-RNP antibody
Associated with mixed connective tissue disease
174
What is rheumatoid factor
An autoantibody (usually IgM) directed against the Fc portion of IgG
175
What is the significance of anti-cyclic citrullinated peptide (anti-CCP) antibodies
Highly specific for rheumatoid arthritis and predictive of disease severity
176
What is the direct antiglobulin test (Coombs test) used for
Detecting antibodies or complement bound to red blood cells in autoimmune hemolytic anemia
177
What is the indirect antiglobulin test used for
Detecting circulating antibodies in serum
178
What is the clinical use of anti-acetylcholine receptor antibody testing
Diagnostic for myasthenia gravis
179
What are anti-neutrophil cytoplasmic antibodies (ANCA)
Autoantibodies directed against components of neutrophil cytoplasm
180
What are the two main ANCA patterns
Cytoplasmic (c-ANCA) and perinuclear (p-ANCA)
181
What diseases are associated with c-ANCA
Wegener’s granulomatosis (granulomatosis with polyangiitis)
182
What diseases are associated with p-ANCA
Microscopic polyangiitis and other vasculitides
183
What is the procedure for the rapid slide test for antinucleoprotein
Mix patient serum with reagent cells on a slide
184
What are common causes of false positive rapid slide test results
Rheumatoid factor
185
How should a positive rapid slide test be confirmed
By indirect immunofluorescence assay (IFA) and further specific antibody testing
186
What is the role of immunosuppressive therapy in autoimmune diseases
To reduce immune system activity and prevent tissue damage
187
What are potential side effects of immunosuppressive therapy
Increased risk of infections
188
What is allergen immunotherapy
Gradual introduction of allergens to build immune tolerance and reduce allergic symptoms
189
What is the mechanism of action of monoclonal antibodies like rituximab in autoimmune diseases
Target and deplete B cells that produce autoantibodies
190
What is molecular mimicry in autoimmunity
Pathogens share epitopes with self-antigens
191
What is the role of regulatory T cells in autoimmunity
Suppress autoreactive immune responses and maintain self-tolerance
192
What is the chronicity pattern of systemic lupus erythematosus
Chronic and irregular with periods of exacerbations and remissions
193
What is the typical age range for onset of systemic lupus erythematosus
Most commonly affects women between puberty and menopause
194
What is the female-to-male ratio in systemic lupus erythematosus
Approximately 9:1 female to male
195
What is the role of ultraviolet (UV) light in lupus
UV light can induce or exacerbate skin lesions and systemic disease by causing DNA damage
196
What is the significance of anti-ribosomal P antibodies in lupus
Associated with neuropsychiatric lupus manifestations
197
What are common neuropsychiatric manifestations of SLE
Seizures
198
What is the typical presentation of lupus nephritis
Proteinuria
199
What is the clinical importance of complement levels in lupus
Decreased complement levels (C3
200
What is the significance of leukopenia in lupus
Reflects immune-mediated destruction or suppression of white blood cells
201
What is the typical skin manifestation of discoid lupus
Well-defined
202
What is the difference between discoid lupus and systemic lupus
Discoid lupus is limited to skin; systemic lupus involves multiple organ systems
203
What is the typical treatment approach for lupus
Immunosuppressive therapy including corticosteroids
204
What is the importance of monitoring autoantibody levels in lupus patients
To assess disease activity and guide treatment decisions
205
What is the role of anti-Ro/SSA antibodies in neonatal lupus
Associated with congenital heart block and cutaneous manifestations in newborns
206
What is the significance of antiphospholipid antibodies in pregnancy
Increased risk of miscarriage
207
What is the typical clinical course of drug-induced lupus
Symptoms resolve upon discontinuation of the offending drug
208
What are the common drugs associated with drug-induced lupus
Hydralazine
209
What is the role of genetic predisposition in lupus
Certain HLA haplotypes increase susceptibility to lupus
210
What is the impact of hormonal factors on lupus
Estrogen may exacerbate lupus symptoms; disease is more common in females
211
What are the common laboratory tests used in lupus diagnosis
ANA
212
What is the significance of a positive ANA test
Highly sensitive but not specific for lupus; used as a screening test
213
What is the gold standard test for ANA detection
Indirect immunofluorescence assay (IFA) on Hep-2 cells
214
What is the clinical relevance of anti-Sm antibodies
Highly specific for lupus diagnosis though less sensitive
215
What is the clinical utility of anti-dsDNA antibodies
Specific for lupus and correlate with renal involvement and disease activity
216
What is the role of complement testing in lupus
Helps monitor disease activity and immune complex-mediated damage
217
What is the typical histologic finding in lupus nephritis
Immune complex deposition in glomeruli leading to inflammation and damage
218
What is the significance of hematologic abnormalities in lupus
Reflect immune-mediated destruction of blood cells contributing to anemia
219
What is the purpose of the direct antiglobulin test in lupus
Detects antibodies bound to red blood cells indicating autoimmune hemolytic anemia
220
What is the clinical importance of antiphospholipid syndrome in lupus
Leads to thrombosis
221
What is the typical presentation of serositis in lupus
Inflammation of pleura
222
What is the role of immunosuppressive drugs in lupus management
Suppress immune response to reduce inflammation and prevent organ damage
223
What are potential complications of long-term immunosuppressive therapy
Infections
224
What is the role of hydroxychloroquine in lupus treatment
Antimalarial drug that reduces disease activity and skin manifestations
225
What is the importance of patient education in lupus management
Helps patients recognize flares
226
What are the common clinical features of discoid lupus
Well-defined; erythematous; scaly plaques that may cause scarring and alopecia
227
How is discoid lupus diagnosed
Biopsy of skin lesions showing interface dermatitis and follicular plugging
228
What is the typical prognosis of discoid lupus
Generally good but can cause permanent scarring; may progress to systemic lupus in some cases
229
What is the typical presentation of drug-induced lupus
Mild symptoms resembling systemic lupus with predominance of musculoskeletal and serosal involvement
230
Which organs are usually spared in drug-induced lupus
Kidneys and central nervous system
231
What happens to drug-induced lupus symptoms after stopping the offending drug
Symptoms usually resolve within weeks to months
232
What is the role of anti-histone antibodies in drug-induced lupus
Present in most cases and useful for diagnosis
233
What is neonatal lupus
Passive transfer of maternal autoantibodies (anti-Ro/SSA and anti-La/SSB) causing transient rash and congenital heart block in newborns
234
What are the clinical manifestations of neonatal lupus
Rash appearing in first weeks of life; possible congenital heart block; hepatobiliary disease; and cytopenias
235
What is the prognosis of neonatal lupus
Rash usually resolves by 6 months; heart block may be permanent and requires pacemaker
236
What is the significance of anti-Ro/SSA and anti-La/SSB antibodies in lupus
Associated with photosensitive skin rash; neonatal lupus; and congenital heart block
237
What environmental factors can exacerbate lupus
Ultraviolet light exposure; infections; stress; and certain medications
238
What is the role of ultraviolet light in lupus pathogenesis
Induces keratinocyte apoptosis and increases autoantigen exposure leading to immune activation
239
What is the genetic predisposition to lupus
Certain HLA alleles and gene polymorphisms increase susceptibility
240
What is the female predominance in lupus attributed to
Hormonal influences; particularly estrogen effects on immune regulation
241
What are the common hematologic abnormalities in lupus
Anemia; leukopenia; lymphopenia; and thrombocytopenia
242
What is the mechanism of anemia in lupus
Chronic inflammation; hemolysis; or bone marrow suppression
243
What is the clinical significance of leukopenia in lupus
Increased risk of infections and indicator of disease activity
244
What are the common renal manifestations of lupus
Proteinuria; hematuria; and varying degrees of glomerulonephritis
245
What is the classification of lupus nephritis
Focal; diffuse; membranous; or proliferative glomerulonephritis based on biopsy
246
What is the treatment approach for lupus nephritis
Immunosuppressive therapy including corticosteroids and cytotoxic agents
247
What are the common neuropsychiatric manifestations of lupus
Seizures; psychosis; cognitive dysfunction; mood disorders
248
What is the role of antiphospholipid antibodies in lupus
Associated with thrombosis; recurrent pregnancy loss; and antiphospholipid syndrome
249
What laboratory tests are used to detect antiphospholipid antibodies
Anticardiolipin antibody; lupus anticoagulant; and anti-beta2 glycoprotein I antibody
250
What is the clinical significance of a positive lupus anticoagulant test
Increased risk of thrombosis despite prolongation of clotting times in vitro
251
What is the role of complement levels in lupus monitoring
Low C3 and C4 levels indicate active disease and immune complex formation
252
What is the significance of anti-dsDNA antibody titers in lupus
Correlate with disease activity; especially renal involvement
253
What is the importance of regular monitoring in lupus patients
To detect flares early; adjust treatment; and prevent organ damage
254
What are the main goals of lupus treatment
Control symptoms; prevent flares; minimize organ damage; and improve quality of life
255
What medications are commonly used in lupus management
NSAIDs; corticosteroids; antimalarials (hydroxychloroquine); immunosuppressants (azathioprine; cyclophosphamide); biologics (rituximab; belimumab)
256
What are the side effects of long-term corticosteroid use
Weight gain; osteoporosis; diabetes; hypertension; increased infection risk
257
What is the role of hydroxychloroquine in lupus
Reduces disease activity; prevents flares; and has a favorable safety profile
258
What lifestyle modifications are recommended for lupus patients
Avoid sun exposure; maintain balanced diet; regular exercise; and avoid smoking
259
What is the prognosis of systemic lupus erythematosus
Improved with modern therapy; 10-year survival >90%
260
What are the major causes of mortality in lupus
Renal failure; infections; cardiovascular disease
261
What is the significance of patient education in lupus management
Empowers patients to recognize symptoms; adhere to therapy; and avoid triggers
262
What is the typical presentation of lupus arthritis
Nonerosive+ symmetric polyarthritis affecting small joints+ causing pain and swelling
263
How does lupus arthritis differ from rheumatoid arthritis
Lupus arthritis is usually nonerosive and less deforming than rheumatoid arthritis
264
What is the characteristic skin manifestation of subacute cutaneous lupus
Annular or papulosquamous lesions often triggered by sun exposure
265
What are common triggers for lupus flares
Ultraviolet light+ infections+ stress+ hormonal changes+ and certain medications
266
What is the role of B cells in lupus pathogenesis
Produce autoantibodies and present antigens to T cells+ contributing to immune dysregulation
267
What is the function of T regulatory cells in lupus
Normally suppress autoreactive immune responses+ their dysfunction contributes to disease
268
What is the significance of apoptotic cell clearance in lupus
Defective clearance leads to persistence of autoantigens and immune activation
269
What is the mechanism of immune complex-mediated tissue damage in lupus
Deposition of antigen-antibody complexes activates complement and inflammatory cells+ causing tissue injury
270
What is the clinical importance of anti-ribosomal P protein antibodies
Associated with lupus psychosis and neuropsychiatric manifestations
271
What is the significance of anti-histone antibodies
Commonly seen in drug-induced lupus but also present in some systemic lupus cases
272
What are the common hematologic complications in lupus
Autoimmune hemolytic anemia+ leukopenia+ thrombocytopenia
273
What is the typical cause of thrombocytopenia in lupus
Autoimmune destruction of platelets
274
What is the role of complement deficiencies in lupus pathogenesis
Genetic deficiencies in early complement components increase susceptibility to lupus
275
What is the clinical relevance of anti-C1q antibodies
Associated with lupus nephritis and disease activity
276
What is the importance of renal biopsy in lupus nephritis
Determines class and guides treatment decisions
277
What are the common classes of lupus nephritis
Class I (minimal mesangial)+ II (mesangial proliferative)+ III (focal)+ IV (diffuse)+ V (membranous)+ VI (advanced sclerosing)
278
What is the typical treatment for class III and IV lupus nephritis
High-dose corticosteroids and immunosuppressive agents such as cyclophosphamide or mycophenolate mofetil
279
What are the clinical features of antiphospholipid syndrome
Recurrent arterial or venous thrombosis+ pregnancy morbidity+ and presence of antiphospholipid antibodies
280
What is the treatment for antiphospholipid syndrome
Long-term anticoagulation with warfarin or heparin during pregnancy
281
What are the neuropsychiatric syndromes associated with lupus
Headache+ mood disorders+ cognitive dysfunction+ seizures+ psychosis+ cerebrovascular disease
282
What is the role of MRI in neuropsychiatric lupus
Detects brain lesions and helps differentiate causes of symptoms
283
What are common cardiac manifestations of lupus
Pericarditis+ myocarditis+ Libman-Sacks endocarditis
284
What is Libman-Sacks endocarditis
Nonbacterial verrucous vegetations on heart valves seen in lupus
285
What are the pulmonary complications of lupus
Pleuritis+ interstitial lung disease+ pulmonary hypertension+ alveolar hemorrhage
286
What is the significance of elevated ESR and CRP in lupus
Markers of inflammation but can be elevated in infections or other conditions
287
What is the clinical use of anti-Ro/SSA and anti-La/SSB antibodies in lupus
Associated with photosensitivity+ subacute cutaneous lupus+ and neonatal lupus
288
What is the role of belimumab in lupus treatment
Monoclonal antibody targeting B-lymphocyte stimulator (BLyS) to reduce B cell activity
289
What are the indications for biologic therapy in lupus
Refractory disease not responding to conventional immunosuppressants
290
What are common side effects of immunosuppressive therapy in lupus
Infections+ cytopenias+ liver toxicity+ gastrointestinal symptoms
291
What is the importance of vaccination in lupus patients
Prevent infections especially when on immunosuppressive therapy
292
What lifestyle advice is important for lupus patients
Avoid sun exposure+ smoking cessation+ stress management+ and regular exercise
293
What is the role of pregnancy management in lupus
Close monitoring due to increased risk of flares and pregnancy complications
294
What are the recommendations for contraception in lupus patients
Use non-estrogen methods due to thrombosis risk with estrogen-containing contraceptives
295
What is the prognosis of neonatal lupus
Rash resolves by 6 months+ congenital heart block may require pacemaker and has significant morbidity
296
What is the significance of family history in lupus
Increased risk among first-degree relatives suggesting genetic predisposition
297
Case Study 1= Patient with malar rash+ arthritis+ and proteinuria - What is the diagnosis?
Systemic lupus erythematosus (SLE)
298
Case Study 1= What autoantibodies support diagnosis?
Positive ANA+ anti-dsDNA+ anti-Sm antibodies
299
Case Study 1= What laboratory findings are expected?
Low complement levels (C3+ C4)+ proteinuria+ hematuria+ cellular casts
300
Case Study 1= What does proteinuria indicate in lupus?
Renal involvement (lupus nephritis)
301
Case Study 2= Patient with photosensitive rash+ dry eyes+ and dry mouth - What is the diagnosis?
Sjögren’s syndrome
302
Case Study 2= What autoantibodies are commonly found?
Anti-SSA (Ro)+ anti-SSB (La)
303
Case Study 2= What is the typical clinical presentation?
Dry eyes (keratoconjunctivitis sicca)+ dry mouth (xerostomia)
304
Case Study 3= Patient with muscle weakness and ptosis - What is the diagnosis?
Myasthenia gravis
305
Case Study 3= What autoantibodies confirm diagnosis?
Anti-acetylcholine receptor antibodies
306
Case Study 3= What is the pathophysiology?
Autoantibodies block acetylcholine receptors at neuromuscular junction causing muscle weakness
307
Case Study 4= Patient with recurrent thrombosis and pregnancy loss - What is the diagnosis?
Antiphospholipid syndrome
308
Case Study 4= What antibodies are associated?
Anticardiolipin antibodies+ lupus anticoagulant+ anti-beta2 glycoprotein I antibodies
309
Case Study 4= What is the treatment?
Long-term anticoagulation therapy (e.g.+ warfarin)
310
What is the clinical relevance of a positive direct antiglobulin test?
Indicates autoimmune hemolytic anemia
311
What is the clinical relevance of a positive indirect antiglobulin test?
Detects circulating antibodies in serum that may cause hemolysis
312
What is the principle of the rapid slide test for antinucleoprotein?
Mix patient serum with reagent cells containing nuclear antigens+ agglutination indicates presence of ANA
313
What specimen is required for the rapid slide test?
Patient serum or plasma
314
What is the appearance of a positive rapid slide test?
Visible clumping or agglutination on the slide
315
What are common causes of false positive rapid slide test results?
Rheumatoid factor+ nonspecific agglutinins+ improper technique
316
How should a positive rapid slide test be confirmed?
By indirect immunofluorescence assay (IFA) and specific antibody testing
317
What precautions should be taken when performing the rapid slide test?
Use proper controls+ avoid contamination+ follow timing strictly
318
What is the clinical significance of a positive rapi
319
What is the purpose of the rapid slide test for antinucleoprotein
To detect antinuclear antibodies (ANA) in patient serum quickly
320
What specimen is required for the rapid slide test
Serum or plasma from the patient
321
What is the principle of the rapid slide test
Patient serum mixed with reagent cells containing nuclear antigens; agglutination indicates ANA presence
322
How is the rapid slide test performed
Mix equal volumes of patient serum and reagent cells on slide; rotate gently; observe for agglutination within minutes
323
What indicates a positive rapid slide test
Visible clumping or agglutination on the slide surface
324
What are common causes of false positive rapid slide test results
Rheumatoid factor+ nonspecific agglutinins+ improper technique
325
How should positive rapid slide test results be confirmed
By indirect immunofluorescence assay (IFA) and specific autoantibody testing
326
What precautions should be taken when performing the rapid slide test
Use proper controls+ avoid contamination+ adhere strictly to timing and technique
327
What is the clinical significance of a positive rapid slide test
Indicates possible autoimmune disease requiring further evaluation and confirmatory testing
328
What is systemic lupus erythematosus (SLE)
A chronic autoimmune disease with multisystem involvement characterized by autoantibody production and immune complex deposition
329
What are the hallmark clinical features of SLE
Malar rash+ arthritis+ photosensitivity+ renal involvement+ hematologic abnormalities
330
What role do autoantibodies play in SLE
Mediate tissue damage through immune complex formation and direct cellular effects
331
What is the importance of complement levels in SLE
Low complement levels indicate active disease and immune complex consumption
332
What are the mainstays of SLE treatment
Immunosuppressive drugs+ corticosteroids+ antimalarials+ biologic agents
333
What is the prognosis for patients with SLE
Improved significantly with modern therapies; 10-year survival exceeds 90%
334
What are common triggers of SLE flares
Ultraviolet light exposure+ infections+ stress+ hormonal changes+ certain medications
335
What is the significance of patient education in managing SLE
Helps patients recognize flares early+ avoid triggers+ and adhere to treatment plans
336
What are the diagnostic criteria for systemic lupus erythematosus
Presence of 4 or more of the 11 ACR criteria including malar rash+ discoid rash+ photosensitivity+ oral ulcers+ arthritis+ serositis+ renal disorder+ neurologic disorder+ hematologic disorder+ immunologic disorder+ positive ANA
337
How do anti-dsDNA antibodies correlate with disease activity
They are highly specific for SLE and correlate with renal involvement and disease flares
338
What is the difference between drug-induced lupus and systemic lupus
Drug-induced lupus is caused by certain medications and usually resolves after drug discontinuation; systemic lupus is chronic and multisystemic
339
Why is monitoring complement levels important in SLE patients
Decreased complement levels indicate active disease and immune complex-mediated tissue damage
340
How can antiphospholipid antibodies affect pregnancy outcomes
They increase risk of miscarriage+ stillbirth+ and thrombosis
341
What is the significance of a positive ANA test
It is sensitive but not specific for SLE; positive results require further specific antibody testing
342
How does ultraviolet light contribute to lupus pathogenesis
By inducing keratinocyte apoptosis and exposing nuclear antigens that trigger autoimmunity
343
What are the common hematologic abnormalities seen in SLE
Anemia+ leukopenia+ thrombocytopenia due to immune-mediated destruction
344
What is the role of hydroxychloroquine in SLE management
Reduces disease activity+ prevents flares+ and has immunomodulatory effects
345
What are the potential complications of long-term corticosteroid therapy
Increased risk of infection+ osteoporosis+ diabetes+ hypertension
