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Pediatric Rheumatology Board Exam > Lupus 12% > Flashcards

Lupus 12% Flashcards

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1
Q

What is a cause of persistently low C4 despite overall improvement in lupus disease activity?

A

C4 null allele

Suspect if C4 can never get above half the normal value for C4 though C3 is improving.

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2
Q

What are common morbidities in lupus?

A

Cardiovascular disease: most common cause of death in patients with lupus
Infection
Drug-induced myopathy (steroids, Plaquenil)

Side effects from chronic steroids:
Diabetes and hyperglycemia
Osteoporosis
Avascular necrosis

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3
Q

What is the differential diagnosis of lupus?

A

MCTD, UCTD
Juvenile dermatomyositis
Sjogren syndrome
Systemic vasculitis (ANCA-associated vasculitis)
TTP/TMA/HUS
Rowell syndrome with lupus, erythema multiforme, +SSA/SSB/RF/ANA
ALPS or other lymphoproliferative disease with secondary autoimmunity
ITP/AIHA
Antiphospholipid syndrome
Autoinflammatory disease like SAVI
Kikuchi disease
Malignancy
Viral infection: parvovirus can cause similar rash to malaria rash except spares nasal bridge, ctypoenias, low complements

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4
Q

What is upregulated in lupus and how does it contribute to the pathophysiology of lupus?

A

Type I interferon (IFN-alpha) stimulates production of innate immune cells (plasmacytoid dendritic cells) that release cytokines causing increased inflammation

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5
Q

Which complement deficiency has the highest percentage of associated lupus (of the complement deficiencies)?

A

C1q deficiency

Lupus is seen in 90% of homozygous cases of C1q deficiency.

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6
Q

What is the most common complement deficiency?

What percentage of patients with this condition are associated with lupus?

What auto-antibody is commonly seen in these patients?

A

C2 deficiency

Lupus seen in 33% of homozygous cases of C2 deficiency.

Higher association with anti-Ro (SSA) Ab.

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7
Q

What complement deficiencies are associated with lupus?

A

C2 deficiency - most common type
C1q deficiency - highest % of associated lupus
C4 allele deficiency

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8
Q

What genetic syndrome is associated with an increased risk of lupus?

A

Klinefelter syndrome (47,XXY)

Usually milder lupus with less risk of lupus nephritis

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9
Q

What are 2 hallmarks of lupus that suggest that adaptive immunity is affected?

A

Autoantibodies and hypergammaglobulinemia

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10
Q

A teenage female with lupus and class III lupus nephritis presents to clinic complaining of new hip and knee pain and limp. What should you evaluate for?

A

Inflammatory arthritis associated with flare
Avascular necrosis - obtain imaging

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11
Q

Is there an increased incidence of autoimmunity in relatives of patients with lupus?

A

Yes

10% of 1st degree relatives also have lupus or another CTD
20-fold increased risk of lupus in siblings

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12
Q

What percentage of monozygous (identical) twins are concordant for lupus?

A

24%

This indicates there are other factors at play, e.g. environmental, epigenetic modifications, etc.

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13
Q

Does UVA or UVB play a larger role in lupus pathophysiology?

A

UVB!

UVB induces keratinocytes to release chemokines and cytokines, which recruits memory T cells and pDCs into the skin, and induces apoptosis and necrosis of keratinocytes, leading to accumulation of nucleic acids and activation of pDCs through TLR signaling. Also, UVB can decrease DNA methylation in PBMCs, which may alter gene expression

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14
Q

What is considered proteinuria in children? When would you call it nephrotic range?

A

Proteinuria in children = spot Up/c >0.2 mg/mg creatinine or by 24h collection >4 mg/mg2/hr

Nephrotic range proteinuria = Up/c >2-3 mg/mg creatinine (spot) or >40 mg/mg2/hr (24h)

Obtain first morning void to eliminate false reading due to possible orthostatic proteinuria

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15
Q

What autoantibody is sensitive for depression and psychosis in lupus but is not specific and can be seen in patients without CNS involvement?

A

Anti-ribosomal P antibodies

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16
Q

What renal manifestation strongly correlates with progression of renal disease in lupus nephritis

A

Tubulointerstitial changes

Tubular epithelial cell alterations, tubular atrophy, interstitial inflammatory infiltrate, interstitial fibrosis. They follow primary glomerular disease.

Predominant tubulointerstitial nephritis is rare in SLE

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17
Q

How does predominant tubulointerstitial nephritis (TIN) present in lupus? Include clinical and histopathology

A

Abnormal renal function
Normal/mild urinary lab findings
Histopathology: mononuclear inflammatory interstitial infiltrate and varying degree of interstitial fibrosis and tubular atrophy
Think bland urine in proportion to abnormal renal function

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18
Q

What histopathologic finding is seen in chronic thrombotic microangiopathy (TMA)?

A

“Onion skinning” of intimal fibroplasia

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19
Q

What is the diagnostic evaluation of pericarditis in lupus?

A

Chest x-ray - enlarged cardiac silhouette if large pericardial effusion present

EKG - elevated ST segments and peaked T waves

Echocardiogram - confirms pericardial effusions and/or pericarditis

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20
Q

A patient with lupus presents with progressive dyspnea, orthopnea, low FVC, normal DLCO on PFTs. What rare condition must be considered?

A

Shrinking lung disease

CXR appears normal or with bibasilar atelectasis and elevated diaphragm

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21
Q

True or False: The arthritis in lupus is typically non-erosive.

A

True. Arthritis in lupus is NON-erosive.

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22
Q

What is Jacoud arthropathy?

A

Tenosynovitis in hands leading to RA-like deformities in lupus

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23
Q

What do you see on histopathology of steroid-induced myopathy?

A

Steroid-induced myopathy shows nonspecific atrophy of type 2b (fast twitch) muscle fibers, absence of inflammatory infiltrate, variations in fiber size with centrally placed nuclei and rarely signs of muscle necrosis.

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24
Q

A 14-year-old female with lupus and class IV lupus nephritis who has received 5 rounds of monthly cyclophosphamide, daily oral steroid, history of steroid pulsing, daily Plaquenil presents with left knee pain. What is on the differential?

A

Avascular necrosis - 2/2 steroid use or vasculitis
Arthritis due to flare
Infection
AMPS/CRPS

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25
What are risk factors for cyclophosphamide-related infertility?
Cumulative dose Age at administration - younger age better
26
True or False: Patients with lupus who are pregnant will experience a flare during pregnancy or postpartum in up to 50% of the time.
True
27
What are risk factors for adverse pregnancy outcomes in patients with lupus?
Hypertension Renal insufficiency Thrombocytopenia Active lupus at conception Presence of APS
28
What medications are safe to use in pregnancy?
Azathioprine Plaquenil Cyclosporine Oral steroids at the lowest possible dose
29
What medications should be avoided in pregnancy?
Methotrexate Leflunomide Cyclophosphamide Mycophenolate mofetil Thalidomide
30
What may cause the thrombocytopenia in neonatal lupus?
Anti-Ro or anti-La antibodies
31
What is the typical skin manifestation in neonatal lupus?
Round, elliptical, erythematous rash occurs in 15-25% - self-resolves, may miss it Rash is photosensitive, can appear with phototherapy Develops in first 6-12 weeks No new lesion beyond 6 months Blueberry muffin rash in minority - due to extra-medullary dermatoerythropoieis
32
What is the typical hematologic manifestations in neonatal lupus?
Anemia and thrombocytopenia - most common Hemolytic anemia Neutropenia Bone marrow failure DIC Thromboses 2/2 APLs
33
What is the first-line treatment for moderate or severe or refractory hematologic manifestations of neonatal lupus?
IVIG 1 gram/kg for 1-2 days
34
What are cardiac manifestations in neonatal lupus?
Bradyarrhythmia due to heart block PR prolongation Myocarditis, endocarditis, ventricular end-myocardial fibroelastosis Congestive heart failure +/- serositis
35
What is the treatment for life-threatening conduction abnormalities of neonatal lupus?
Pacemaker for life-threatening conduction abnormalities
36
In women who have SLE with positive anti-Ro or anti-La Ab, what percentage of the children will have neonatal lupus?
1-10%
37
What is the rate of recurrence of complete heart block (CHB) in pregnancies after the birth of a child with CHB?
15-17%
38
What is recommended to prevent neonatal lupus?
Plaquenil - inhibit activation of intracellular toll-like receptors (TLRs) including TLR-7 which can lead to fibrosis Primary prevention with steroid or IVIG is NOT recommended
39
What are the HLA associations in neonatal lupus?
HLA-DQ and DR (DRB) important in production of SSA/SSB Ab
40
What APL is the most important acquired risk factor for thrombosis?
LUPUS ANTICOAGULANT correlates better with occurrence of thromboembolic events than ACL or b2GPI and is the most important acquired risk factor for thrombosis.
41
What causes lupus anticoagulant hypoprothrombinemia syndrome (LAHS)?
Antiprothrombin Ab that causes rapid depletion of plasma prothrombin and results in hemorrhage
42
How do you treat lupus anticoagulant hypoprothrombinemia syndrome (LAHS)?
Needs aggressive treatment - IVMP pulsing, PLEX, cyclophosphamide, ?IVIG, ?rituximab Avoid rapid shifts in BP, treat hypertension slowly
43
True or false? Venous thrombosis much more common in primary APS and occurs in 30% of peds APS
FALSE Arterial thrombosis much more common in primary APS and occurs in 30% of peds APS
44
What is Sneddon syndrome?
Stroke or transient neurological symptoms with livedo reticularis is associated with APLs in ~50% of cases
45
What congenital abnormality in combination with APLs is a significant risk factor for ischemic stroke?
Factor V Leiden and APLs are significant risk factors for ischemic stroke in kids
46
What hematologic finding is more common in SLE with secondary APS?
Thrombocytopenia
47
What is the significance of livedo reticularis in APS?
Livedo reticularis – particularly livedo racemosa variant – major clinical feature of APS strongly a/w arterial and microangiopathic subtypes
48
In a patient presenting with Raynaud phenomenon, what auto-antibodies should you check?
APLs Raynaud phenomenon can be associated with APLs - 36% of primary Raynaud and 30% of secondary Raynaud had at least 1 APL, so one should check APLs in patients presenting with Raynaud
49
What is one of the hallmarks of CAPS and is present in 70% of patients with CAPS?
Thrombocytopenia
50
What infections are associated with APS?
APS most frequently a/w VZV, parvovirus B19, HIV, Strep, and Staph, Gram negative, Mycoplasma pneumonia
51
What is the risk of developing thrombosis in otherwise healthy, asymptomatic children with incidental +APL?
Low
52
How do APLs cause problems?
aPL promote complement activation, inhibit physiologic anticoagulants (activated protein C, antithrombin, and annexin A5 anticoagulant shield), impair fibrinolysis, increase procoagulant function of platelets, endothelial cells, and leukocytes/monocytes
53
With +APLs, which antibodies (IgG vs IgM) are the most pathogenic?
IgG APLs are the most pathogenic.
54
What is typically inflamed in Sjogren syndrome?
Sjogren syndrome presents with inflammation of exocrine glands
55
Which rheum condition can present with a purely sensory neuropathy?
Sjogren syndrome
56
What does minor salivary gland biopsy of lower lip show in Sjogren syndrome?
lymphocytic sialadenitis
57
What are classic serologic findings associated with MCTD?
Anti-U1-snRNP - 70 kDpolypeptide appears to be dominant autoantigen Anti-U1-RNA HLA-DR4
58
What is the simple pathophysiology of TTP?
A severe functional deficiency of ADAMTS13
59
If you are evaluating for drug induced lupus, exposure to the following medications should make you consider testing for ANCA as well?
Minocycline, hydralazine, propylthiouracil, methimazole
60
What autoantibody is classic for drug-induced lupus? Especially with what drugs?
Anti-histone Ab Minocycline, PTU, and statins
61
What are common drug triggers for drug-induced lupus?
Hydralazine, procainamide, isoniazid, methyldopa, quinidine, minocycline, and chlorpromazine In children, most common triggers are anti-epileptic drugs.
62
What medications have been implicated in suspected drug-induced lupus with lupus nephritis?
Hydralazine, sulfasalazine, PTU, and anti-TNFa therapy Renal disease in drug-induced lupus is rare and usually due to a necrotizing glomerulonephritis with little or no immune complex deposition, although an immune complex-mediated glomerulonephritis can occur
63
What is the classic pentad of TTP?
Microangiopathic hemolytic anemia (MAHA) Thrombocytopenic purpura Fever Neurologic abnormalities: headache, confusion, coma, seizure, stroke, transient focal defects Renal disease: proteinuria, moderate AKI
64
What is the first line treatment for TTP?
Plasmapheresis IV methylprednisolone pulsing Rituximab for refractory
65
Which inherited complement component deficiency is the strongest genetic risk factor for systemic lupus erythematosus as well as a strong predictor of disease severity?
C1q deficiency Almost all (90%) of homozygous C1q deficient patients develop SLE and it is the strongest known genetic risk factor. In addition, disease onset is earlier and tends to be more severe. Though C4 and C2 deficient patients are also at risk of developing SLE, the disease association and severity is not as strong as with C1q deficiency.
66
What is the most common inherited complement component deficiency that is a risk factor for systemic lupus erythematosus?
C2 deficiency Most common complement deficiency Lupus is seen in 33% of homozygous cases Higher association with anti-Ro Ab
67
What is another inherited complement component deficiency is a risk factor/is associated with lupus?
C4 deficiency
68
What is the differential diagnosis of persistent bilateral parotid gland swelling?
Sjӧgren syndrome Sarcoidosis Chronic infection (HIV, hepatitis C virus) IgG4-related disease (IgG4-RD)
69
What is the classic histologic description of IgG4-related disease?
Storiform (haphazard and swirling pattern) fibrosis, obliterative phlebitis, and a lymphoplasmacytic infiltrate
70
A salivary gland biopsy is obtained in a teenage female and shows scattered lymphoid cells. What is the diagnosis?
Normal age-related salivary gland
71
What is the classic histologic description of Sjӧgren syndrome?
The prototypic finding on salivary gland biopsy in Sjӧgren syndrome is at least one focus (collection of > 50 cells) of lymphocytic infiltrate.
72
A patient presents with chronic salivary or lacrimal gland swelling in combination with pancreatitis. What should be high on the differential diagnosis?
IgG4 related disease
73
What viral infection can cause a red rash on cheek, cytopenias, polyarthritis, and low complements that may mimic lupus?
Parvovirus
74
A teenage female with lupus and positive APLs presents with new onset severe headache that does not respond to Motrin or Tylenol. She is receiving IV steroid pulsing for active lupus. What is the differential for this headache?
Stroke/vascular event Infection Intracranial hypertension PRES - headache, sz, AMS, visual loss
75
What is the most effective treatment for lupus psychosis?
Cyclophosphamide
76
What neuropsychological lupus symptoms are associated with positive anti phospholipid antibodies?
Thrombotic phenomenon Chorea Transverse myelitis
77
Which lupus rashes are nonscarring?
Malar rash Subacute cutaneous lupus Tumid lupus Discoid lupus is scarring.
78
A patient with lupus has livedo reticularis. What lab abnormality is this rash associated with?
APLs
79
What is the classic histologic finding in skin lesions in lupus?
Disruption of the dermal-epidermal junction aka interface dermatitis There are ICs by IF at DEJ in normal and lesion all skin. There is infiltration of T cell in the skin and peri vascular. Mucin deposits typically found. Fibrinoid degeneration of connective tissue.
80
What is the treatment of skin lesions in lupus?
Sun protection Topical steroids and/calcineurin inhibitors Plaquenil Second line could be azathioprine or dapsone
81
What is the most common cardiac manifestation in lupus?
Pericarditis
82
What is the most common valvular finding in lupus?
Left sided valve thickening and regurgitation
83
What is Libman-Sacks endocarditis?
Endocarditis in lupus with sterile, small, verrucous valve lesions, typically MV, chordae tendinae, and/or endocardium. It is associated with longer disease duration, higher lupus disease activity, and APLs.
84
How does the risk for coronary artery disease in lupus compare to controls?
Risk of CAD is 4-8x higher Young women with lupus are 50x more likely to have a heart attack
85
What were the findings in the APPLE study in lupus?
Atherosclerosis Prevention in Pediatric Lupus Erythematosus Atorvastatin is safe in pediatric lupus and lowers total cholesterol, LDL, Tgl, and CRP However, has no significant effect on progression to carotid media intima thickening
86
What auto antibody is associated with congenital heart block in neonatal lupus?
SSA (anti-Ro)
87
What are common bacterial organisms that cause lung infections in lupus?
Gram negative rods Gram positive cocci Chlamydia pneumoniae Mycoplasma pneumoniae
88
What is Budd-Chiari syndrome (BCS) Chiari syndrome and one rheum condition(s) is it seen in?
Acute hepatic vein thrombosis associated with APS with secondary cirrhosis and ascites. Can be seen as a liver manifestation in lupus.
89
What percentage of babies with mothers with SSA/Ro and/or SSB/La antibodies will develop congenital heart block? How can you reduce the risk?
2%, Hydroxychloroquine Congenital heart block develops in the offspring of approximately 2% of mothers with SSA/Ro and/or SSB/La antibodies. Hydroxychloroquine use during pregnancy by mothers that are SSA/Ro and or SSB/La antibody positive may reduce the risk of congenital heart block in the neonate.
90
An asymptomatic mother of an infant with neonatal lupus has what chance of developing lupus? what is the chance of developing autoimmune disease?
10-20 % develop lupus 50 % develop autoimmune disease (not all are lupus)
91
What drugs are associated with drug induced ANCA associated vasculitis?
Minocycline, hydralazine, PTU, methimazole Will have more severe kidney involvement and may require cytotoxic treatment.
92
How quickly should drug induced lupus resolve once causative agent is stopped?
Weeks to months. Less than 6 months. Re-exposure to the drug can cause return of symptoms within 1-2 days and with each exposure the latency period decreases and symptom severity increases. Auto-antibody titers decrease gradually and can persist for months to years after resolution of clinical symptoms.
93
How common is lupus nephritis amount patients with childhood onset lupus and when does it typically occur?
More than half of childhood-onset SLE patients will have nephritis. and it happens early (within first year) Link with anti-dsDNA antibodies and proliferative lupus nephritis (but not necessary) Of those with childhood SLE and lupus nephritis, 83% developed nephritis within the first year after diagnosis of SLE; most of these patients had symptoms at initial presentation.
94
How do the rates of neuropsychiatric lupus compare in kids vs adults with lupus?
Not known, conflicting reports There is no clear evidence of differential rates of neuropsychiatric disease in childhood-onset vs adult-onset SLE. Studies have shown conflicting results, with some studies suggesting higher rates in children. most patients with neuropsychiatric SLE will have features develop within the first year after diagnosis.
95
What lupus symptoms happen more often in kids than in adults?
nephritis, thrombocytopenia, and hemolytic anemia studies have demonstrated that children with SLE do have higher rates of nephritis, thrombocytopenia, and hemolytic anemia as compared to their adult counterparts.
96
Which medicine do black and hispanic lupus patients for induction therapy of class III/V disease respond better to cyclophosphamide or mycophenolate mofetil?
MMF better in black and hispanic Although prospective comparisons of mycophenolate mofetil (MMF) use in different ethnic groups have not been done, post hoc analysis of an international clinical trial in adults has suggested a more favorable response in African Americans. The Aspreva Lupus Management Study compared MMF to monthly cyclophosphamide for induction therapy of class III/V disease. black patients responded better to MMF than cyclophosphamide (54% and 40%, respectively). A similar difference was seen in Hispanic patients (61% and 39%, respectively). This study was specific to the treatment of adults with lupus nephritis and the findings may not be applicable to children or to the treatment of SLE as a whole.
97
What lupus rash has this histology: subepidermal bulla with a neutrophil-predominant infiltrate in the superficial dermis and no evidence of interface dermatitis How do you treat it?
Bullous lupus, treat with dapsone Bullous lupus, also known as acquired SLE-associated bullous dermatoses, is a rare and distinct complication of SLE. Affected patients can develop a vesicular or bullous eruption that primarily affects the skin of the upper body but may include any site, including the oral mucosa. Manifestations can range in severity, occasionally mirroring that of a burn patient, but scarring does not typically occur. Diagnosis of bullous lupus is based on clinical presentation, skin biopsy, a positive antinuclear antibody titer or a diagnosis of SLE, and exclusion of other blistering diseases. Histology reveals subepidermal blistering and a neutrophil-predominant inflammatory infiltrate in the upper dermis with dermal edema. Direct immunofluorescence demonstrates deposition of IgG, IgA, IgM, and/or complement at the basement membrane. Dapsone is often an effective treatment for this subset of patients.
98
What lupus rash has this histology: interface dermatitis with a lymphohistiocytic infiltrate in the superficial dermis and dermal mucin deposition
subacute cutaneous lupus rash Typically, patients with subacute cutaneous lupus erythematosus present with erythematous lesions that are often raised (papular or annular) with varying degrees of scale.
99
What lupus rash has this histology: Perivascular infiltrates of mononuclear cells with hyaline fat necrosis and lymphocytic vasculitis
lupus profundus (lupus panniculitis), a rare cutaneous manifestation of SLE. Lupus profundus typically presents with firm, indurated, erythematous, tender nodules; over time these lesions can resolve leaving behind deep depressed areas of lipoatrophy.
100
What lupus rash has this histology: interface dermatitis with apoptotic keratinocytes, vacuolization of the basal cell layer of the epidermis, lymphohistiocytic infiltrate in the superficial dermis, and dermal mucin deposition.
acute cutaneous lupus erythematosus
101
What lupus rash has this histology: follicular plugging, hyperkeratosis, and mononuclear cell infiltration most predominantly in the periappendageal and perivascular areas
chronic discoid lesions appear as a thickened, hypertrophied, plaque-like lesion that commonly results in scarring.
102
What medicine used to treat rheumatoid arthritis, is not effective with the arthritis associated with lupus?
rituximab must use caution with TNF Interestingly, rituximab (B-cell–depleting therapy), which has been successfully used in the treatment of refractory rheumatoid arthritis in adults, does not induce arthritis remission in patients with lupus. Biologics, such as monoclonal antibody therapy against tumor necrosis factor-α (anti-TNF), have been revolutionary in the treatment of adult and pediatric rheumatoid and idiopathic arthritis. However, caution must be exercised in their use to treat arthritis in patients with lupus. Anti-TNF agents can cause a lupus-like syndrome that can present with serologic and clinical manifestations, such as rash and arthritis. To date, a few case series have demonstrated the efficacy of anti-TNF therapy in controlling arthritis in patients with lupus, and one series demonstrated a quick relapse rate on withdrawal of anti-TNF therapy.
103
What disease manifestations are much less commonly seen in MCTD (compared to lupus)?
Severe renal and CNS disease, not common in MCTD Mixed connective tissue disease is characterized clinically by the almost universal presence of Raynaud phenomenon, as well as arthritis, myositis (without weakness), swollen digits or hands, and eventual development of interstitial lung disease, cardiac disease, and gastrointestinal dysmotility. Mixed connective tissue disease is characterized serologically by very high–titer antinuclear antibody and anti-ribonucleoprotein antibody without antibodies to double-stranded DNA, Smith, SS-A, and Scl-70.
104
What are some differences between the skin disease seen with lupus and MCTD?>
MCTD skin disease primarily consists of RP and swollen digits Malar and other photosensitive rashes, as seen with SLE, are rare in MCTD.
105
What is different about the arthritis of lupus compared to the arthritis of MCTD?
Lupus arthritis typically nondeforming MCTD arthritis can have erosions
106
What are symptoms suggestive of adrenal insufficiency in a patient treated w chronic steroids?
Fever Fatigue Anorexia, nausea, vomiting Abdominal pain Weight loss Hypotension Tachycardia Hyponatremia Myalgias, arthralgias Headache, lethargy, delirium Emotional lability Symptoms are usually related to glucocorticoid deficiency Obtain morning cortisol and ACTH level with ACTH stimulation A tertiary adrenal insufficiency due to interference with corticotropin releasing hormone from the hypothalamus. Low CRH, low ACTH, low cortisol.

Pediatric Rheumatology Board Exam (21 decks)

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