61.2: Lupus Erythematosus Flashcards
What are the laboratory testing indicators for Acute Cutaneous Lupus Erythematosus (ACLE)?
High-titer ANA, anti-dsDNA, anti-Smith antigen, hypocomplementemia, hypergammaglobinemia, cytopenias, decreased kidney function, and urine changes reflect disease activity.
What are the laboratory testing indicators for Subacute Cutaneous Lupus Erythematosus (SCLE)?
Indicators include anti-Ro/SS-A (70-90%), anti-La/SS-B (30-50%), ANA (60-80%), rheumatoid factor (approximately 33%), false-positive VDRL and RPR (7-33%), anticardiolipin (10-16%), antithyroid (18-44%), anti-Smith antigen (10%), anti-dsDNA (10%), and anti-U1 ribonucleoprotein (10%).
What is the significance of anti-Ro/SS-A antibodies in SCLE?
Anti-Ro/SS-A antibodies are present in 70-90% of SCLE cases and are associated with the disease’s pathogenesis.
What are the laboratory findings in SCLE?
Findings include anti-Ro/SS-A (70-90%), anti-La/SS-B (30-50%), ANA (60-80%), rheumatoid factor (33%), false-positive VDRL and RPR (7-33%), anticardiolipin (10-16%), and antithyroid antibodies (18-44%).
What are the key laboratory tests and their significance in diagnosing Acute Cutaneous Lupus Erythematosus (ACLE)?
Key laboratory tests for ACLE include high-titer ANA, anti-dsDNA, anti-Smith antigen, hypocomplementemia, hypergammaglobinemia, and indicators of kidney function and urine changes reflect disease activity.
What laboratory findings are typically associated with Subacute Cutaneous Lupus Erythematosus (SCLE) and their prevalence?
Typical laboratory findings in SCLE include anti-Ro/SS-A (70-90%), anti-La/SS-B (30-50%), ANA (60-80%), rheumatoid factor (approximately 33%), false-positive VDRL and RPR (7-33%), anticardiolipin (10-16%), antithyroid (18-44%), anti-Smith antigen (10%), anti-dsDNA (10%), and anti-U1 ribonucleoprotein (10%).
How do the laboratory findings differ between ACLE and SCLE in terms of disease activity indicators?
ACLE shows high-titer ANA, anti-dsDNA, anti-Smith antigen, hypocomplementemia, hypergammaglobinemia, and indicators of kidney function reflecting disease activity, while SCLE has specific autoantibodies like anti-Ro/SS-A and anti-La/SS-B with less emphasis on kidney function changes.
What are the common laboratory findings in patients with Discoid Lupus Erythematosus (DLE)?
Common findings include low-titer ANA in 30-40% of patients, higher ANA levels in 5% of patients with overt SLE, anti-ssDNA antibodies, distinctly uncommon anti-dsDNA antibodies, precipitating antibodies to U1 ribonucleoprotein, low levels of anti-Ro/SS-A antibodies, low-grade anemia, and modest leukopenia.
What histopathological features are characteristic of Subacute Cutaneous Lupus Erythematosus (SCLE)?
Histopathological features include interface dermatitis with vacuolar alteration of basal keratinocytes, pronounced epidermal atrophy, dermal edema, prominent mucin deposition, and sparse mononuclear cell infiltration.
What is the significance of the lupus band test in diagnosing cutaneous lupus erythematosus?
The lupus band test detects immunoglobulins and complement at the dermal-epidermal junction. In ACLE, 60-100% show a lupus band; in SCLE, a ‘dust-like particle’ pattern of IgG deposition is more specific.
What is the significance of the lupus band test in diagnosing ACLE?
The lupus band test shows IgG, IgA, IgM, C3, and other complement components deposited at the dermal-epidermal junction, positive in 60-100% of ACLE cases.
What are the histopathological features of CCLE?
CCLE shows hyperkeratosis, variable atrophy, thickened basement membrane, dense mononuclear cell infiltrate, and melanophages.
What are the histopathological features of SCLE?
SCLE presents as interface dermatitis with vacuolar alteration of basal keratinocytes, pronounced epidermal atrophy, dermal edema, prominent mucin deposition, and sparse mononuclear cell infiltration.
What are the histopathological differences between SCLE and CCLE?
SCLE shows interface dermatitis and pronounced epidermal atrophy, while CCLE shows hyperkeratosis and dense mononuclear cell infiltrate extending into the deeper dermis.
What are the histopathological features of ACLE?
ACLE shows sparse lymphohistiocytic infiltrate, increased neutrophils, focal vacuolar alteration of basal keratinocytes, telangiectases, extravasation of erythrocytes, and individually necrotic keratinocytes.
What are the clinical features of LE profundus?
LE profundus shows immunoglobulin and complement deposits in blood vessel walls of the deep dermis and subcutis, with ANA positive in 70-75% of cases, but anti-dsDNA is rare.
What is the significance of the lupus band test in CCLE?
In CCLE, the lupus band test is more frequently positive in lesions on the head, neck, and arms (80%) than on the trunk (20%).
What are the laboratory findings in DLE?
Findings include low-titer ANA in 30-40%, anti-ssDNA (not uncommon), anti-dsDNA (distinctly uncommon), and sometimes precipitating antibodies to U1 ribonucleoprotein.
What are the common laboratory findings in patients with Discoid Lupus Erythematosus (DLE)?
Common findings include low-titer ANA in 30-40% of patients, higher ANA levels in 5% of patients with overt SLE, anti-ssDNA antibodies, distinctly uncommon anti-dsDNA antibodies, precipitating antibodies to U1 ribonucleoprotein, low levels of anti-Ro/SS-A antibodies, and low-grade anemia.
How does the histopathological presentation of Subacute Cutaneous Lupus Erythematosus (SCLE) differ from that of Discoid Lupus Erythematosus (DLE)?
SCLE shows pronounced epidermal atrophy and sparse mononuclear cell infiltration, while DLE shows hyperkeratosis and dense mononuclear cell infiltration.
What is the clinical significance of the presence of a lupus band in patients with Cutaneous Lupus Erythematosus (CLE)?
The presence of a lupus band indicates strong association with the disease, being positive in 60-100% of ACLE patients, and more specific patterns in SCLE.
What is the significance of the presence of 3 or more immunoreactants in non-lesional skin for SLE diagnosis?
The presence of 3 or more immunoreactants correlates positively with the risk for developing lupus nephritis and indicates a high specificity for SLE.
What are the ominous prognostic signs associated with SCLE?
Ominous prognostic signs for SCLE include hypertension, nephritis, systemic vasculitis, and CNS disease.
What is the typical clinical course of ACLE?
ACLE can be localized or generalized, with flares and remissions associated with underlying SLE. The survival rate is approximately 80-95% at 5 years and 70-90% at 10 years.
What percentage of patients with SCLE develop active SLE, and what are some associated risk factors?
Approximately 10% of patients with SCLE develop active SLE, including lupus nephritis. Risk factors include papulosquamous SCLE, localized ACLE, high-titer ANA, leukopenia, and/or antibodies to dsDNA.
What are the recommended local therapy measures for sun protection in patients with CLE?
Recommended measures include wearing tightly woven clothing, broad-brimmed hats, and using broad-spectrum, water-resistant sunscreens (SPF ≥30) with an efficient ultraviolet A blocking agent.
What are the ominous prognostic signs in ACLE?
Ominous signs include hypertension, nephritis, systemic vasculitis, and CNS disease.
What are the clinical features of ACLE?
ACLE can be localized or generalized, flaring and abating with underlying SLE. Five-year survival rates are 80-95%, and 10-year rates are 70-90%.
A patient with SCLE presents with intermittent recurrences and long-term remissions. What are the risk factors for developing active SLE in such patients?
Risk factors include papulosquamous SCLE, localized ACLE, high-titer ANA, leukopenia, and/or antibodies to dsDNA.
What is the recommended sun protection strategy for patients with CLE?
Patients should wear tightly woven clothing, broad-brimmed hats, and regularly use broad-spectrum, water-resistant sunscreens (SPF ≥30) with an efficient ultraviolet A blocking agent.
What are the clinical features of reticulated erythematosus mucinosis?
It presents as a reticulated array of macules and/or papules on the upper chest and back and is related to LE tumidus.
What are the potential complications of untreated classic DLE?
Untreated classic DLE can lead to indolent progression to large areas of cutaneous dystrophy, scarring alopecia, and occasionally squamous cell carcinoma.
What are the clinical features of SCLE?
SCLE is characterized by intermittent recurrences, long-term remissions, or unremitting cutaneous disease. About 10% of patients develop active SLE, including lupus nephritis.
What are the clinical features of CCLE?
CCLE can lead to large areas of cutaneous dystrophy, scarring alopecia, and occasionally squamous cell carcinoma. Spontaneous remission is rare.
What are the clinical implications of the presence of 3 or more immunoreactants in nonlesional skin for patients with SLE?
The presence of 3 or more immunoreactants correlates positively with the risk for developing lupus nephritis, indicating a higher likelihood of systemic involvement in SLE.
What is the significance of the survival rates for ACLE and SCLE in terms of prognosis?
The survival rates for ACLE are 80-95% at 5 years and 70-90% at 10 years, while SCLE patients may experience intermittent recurrences and have a 10% chance of developing active SLE.
How does the management of ACLE differ from that of SCLE and CCLE?
Management of ACLE typically involves systemic immunosuppressive measures, while SCLE and CCLE prefer nonimmunosuppressive treatment modalities.
What are the prognosis rates for ACLE and SCLE?
ACLE are 80-95% at 5 years and 70-90% at 10 years, while SCLE patients may experience intermittent recurrences and have a 10% chance of developing active SLE, including lupus nephritis.
What are the recommended local therapy strategies for sun protection in patients with CLE?
Recommended strategies include wearing tightly woven clothing, broad-brimmed hats, and using broad-spectrum, water-resistant sunscreens (SPF ≥30) with effective ultraviolet A blocking agents such as avobenzone or micronized zinc oxide.
What are the recommended topical glucocorticoids for treating Cutaneous Lupus Erythematosus (CLE)?
- Triamcinolone acetonide 0.1% for the face, applied 2x daily for 2 weeks followed by a 2-week rest.
- Superpotent topical agents like clobetasol propionate 0.05% or betamethasone dipropionate 0.05% are also effective.
- Topical calcineurin inhibitors such as pimecrolimus 1% cream and tacrolimus 0.1% ointment can be used.
What is the role of antimalarials in the treatment of Cutaneous Lupus Erythematosus (CLE)?
- Hydroxychloroquine sulfate (Plaquenil): 6 to 6.5 mg/kg of lean body mass daily; may take 2-3 months for therapeutic benefit.
- If no response after 8-12 weeks, add quinacrine 100mg/day (no risk for retinopathy).
- Chloroquine diphosphate (Aralen): 3 to 4 mg/kg/day, more retinotoxic than HCQ.
- Quinacrine: 100mg/day, with side effects including headache and hematologic toxicity.
What are the potential side effects of quinacrine when used for CLE treatment?
- Headache
- GI intolerance
- Hematologic toxicity
- Pruritus
- Lichenoid drug eruptions
- Mucosal or cutaneous pigmentary deposition
- Yellow discoloration of the skin and sclera in fair-skinned individuals (reversible)
- Significant hemolysis in G6PD deficient patients.
What precautions should be taken before starting antimalarial therapy for CLE?
- A pretreatment ophthalmologic examination should be performed due to the risk of antimalarial retinopathy.
- CBC, liver, and renal function tests should be conducted before treatment and repeated 4 to 6 weeks after therapy initiation, and every 4 to 6 months thereafter.
What are the recommended dosages for dapsone in the treatment of refractory CLE?
- Dapsone: Start with 25 mg by mouth twice daily, which can be increased up to 200 to 400 mg/day.
- Monitor for dose-related hemolysis and methemoglobinemia, especially in G6PD deficient patients.
What are the adverse effects of chloroquine in CLE treatment?
Chloroquine is more retinotoxic than hydroxychloroquine and should not be used simultaneously with it. Doses must be adjusted for decreased renal or hepatic function.
What are the systemic side effects of thalidomide in CLE treatment?
Side effects include teratogenicity, sensory neuropathy, somnolence, constipation, and thromboembolism. Neuropathy correlates with total treatment time.
How should hydroxychloroquine sulfate be administered for CLE, and what are the precautions?
Hydroxychloroquine sulfate should be given at 6 to 6.5 mg/kg of lean body mass daily, either once daily or in two divided doses to prevent GI side effects. Pretreatment ophthalmologic examination is required due to the rare risk of antimalarial retinopathy.
A patient with CLE is unresponsive to hydroxychloroquine after 8-12 weeks. What is the next step in treatment?
Add quinacrine 100 mg/day, as it has no risk for retinopathy. If no control is achieved after 4-6 weeks with HCQ+Quinacrine, consider switching to chloroquine.
What are the potential side effects of quinacrine in CLE treatment?
Side effects include headache, GI intolerance, hematologic toxicity, pruritus, lichenoid drug eruptions, mucosal or cutaneous pigmentary deposition, yellow discoloration of the skin and sclera (reversible), and significant hemolysis in G6PD-deficient patients.
What is the role of topical calcineurin inhibitors in CLE treatment?
- Pimecrolimus 1% cream and tacrolimus 0.1% ointment have demonstrated efficacy in treating ACLE, DLE, and SCLE.
- Pimecrolimus 1% cream has shown equal efficacy with betamethasone valerate 0.1% cream in treating facial DLE.
What are the risks associated with thalidomide in CLE treatment?
Risks include teratogenicity, sensory neuropathy (25-75% of patients develop peripheral neuropathy), somnolence, constipation, and thromboembolism. Neuropathy correlates with total treatment time, so short courses are preferred.
What are the systemic side effects of isotretinoin and acitretin in CLE treatment?
Side effects include teratogenicity, dryness, hyperlipidemia, and potential breakthrough of CLE activity.
What is the role of topical tacrolimus in CLE treatment?
Topical tacrolimus 0.3% compounded in clobetasol propionate 0.05% is used for recalcitrant CLE.
What are the potential side effects of dapsone in CLE treatment?
Side effects include dose-related hemolysis, methemoglobinemia (especially in G6PD-deficient patients), and the need for regular CBC and liver function tests.
What are the recommended topical glucocorticoids for treating Cutaneous Lupus Erythematosus (CLE) and their application frequency?
- Triamcinolone acetonide 0.1%: Apply 2 times daily for 2 weeks followed by a 2-week rest.
- Superpotent topical agents (e.g., clobetasol propionate 0.05%, betamethasone dipropionate 0.05%): Produce the greatest benefit in CLE.
What is the role of topical calcineurin inhibitors in the treatment of CLE?
- Pimecrolimus 1% cream and tacrolimus 0.1% ointment have shown efficacy in treating ACLE, DLE, and SCLE.
- Topical calcineurin can be used daily during rest periods.
What are the systemic therapy options for Cutaneous Lupus Erythematosus (CLE) and their potential side effects?
- Hydroxychloroquine sulfate (Plaquenil): 6 to 6.5 mg/kg of lean body mass daily; delayed onset of therapeutic benefit, risk of retinopathy.
- Chloroquine diphosphate (Aralen): 3 to 4 mg/kg/day; more retinotoxic than HCQ.
- Quinacrine: 100 mg/day; side effects include headache, GI intolerance, and hematologic toxicity.
- Dapsone: 25 mg twice daily, can increase to 200-400 mg/day; risk of hemolysis and methemoglobinemia.
What precautions should be taken before starting antimalarial therapy for CLE?
- A pretreatment ophthalmologic examination should be performed due to the risk of antimalarial retinopathy.
- Regular monitoring of CBC and liver/renal function tests is essential.
What are the potential adverse effects of Thalidomide when used for refractory CLE?
- Thalidomide: 50 to 200 mg/day; potential adverse effects include teratogenicity, sensory neuropathy (25-75% of patients may develop peripheral neuropathy), and thromboembolism.
What are the immunosuppressive options for antimalarial refractory disease in patients with cutaneous lupus erythematosus (CLE)?
- Systemic Glucocorticoids: IV methylprednisolone for severe cases. Prednisone 20 to 40 mg/day with gradual reduction to avoid avascular necrosis.
- Traditional Immunosuppressives: Azathioprine (1.5 to 2 mg/kg/day), Mycophenolate mofetil (2.5 to 3 g/day), Methotrexate (7.5 to 25 mg/week).
- Biologics: Anti-TNF medications, B-lymphocyte stimulator inhibitors, Rontalizumab, sifalimumab, Anifrolumab, Tofacitinib, and ruxolitinib.
What are the surgical and cosmetic therapy options for patients with cutaneous lupus erythematosus (CLE)?
- Hair Transplantation and Dermabrasion: With risks due to CLE’s tendency for nonspecific mechanical trauma.
- Scar Revision Techniques: If on maintenance systemic therapy.
- Laser Treatments: Argon and pulsed dye laser, Erbium:YAG or Fraxel CO2 for resurfacing atrophic scars.
- Autologous Fat Transplant: For atrophic scarring and burned out lupus panniculitis.
- Avoid Collagen and Foreign Cosmetic Material Injections: Due to potential complications.
What preventive measures are recommended for patients with cutaneous lupus erythematosus (CLE)?
- Physical protection from the sun: Use of broad spectrum sunscreens.
- Cessation of cigarette smoking: Encouraged to discontinue smoking as it is associated with CLE.
What systemic therapy is recommended for severe and symptomatic CLE?
IV methylprednisolone is recommended for severe and symptomatic CLE.
What is the role of tofacitinib in CLE treatment?
Tofacitinib (JAK 1/3 inhibitor) has shown clinical benefit in CLE and cutaneous dermatomyositis in case reports.
What is the role of belimumab in CLE treatment?
Belimumab is a fully human monoclonal antibody directed against BLyS, which is FDA-approved for SLE.
What are the recommended preventive measures for CLE patients?
Preventive measures include physical protection from the sun, use of broad-spectrum sunscreens, and discontinuation of cigarette smoking.
What is the role of methotrexate in CLE treatment?
Methotrexate (7.5 to 25 mg orally per week) is effective for severe refractory CLE.
What are the adverse effects of systemic glucocorticoids in CLE treatment?
Adverse effects include steroid-induced bone loss, avascular bone necrosis, and adrenal suppression.
What is the role of azathioprine in CLE treatment?
Azathioprine (1.5 to 2 mg/kg per day orally) can play a glucocorticoid-sparing role in CLE treatment.
What are the adverse effects of lenalidomide in CLE treatment?
Lenalidomide has a lower rate of peripheral neuropathy compared to thalidomide but shares similar efficacy in CLE treatment.
What is the role of mycophenolate mofetil in CLE treatment?
Mycophenolate mofetil (2.5 to 3 g divided twice daily orally) provides more specific inhibition of the de novo pathway in lymphocytes.
What are the risks of using anti-TNF medications in CLE treatment?
Anti-TNF medications are used for recalcitrant CLE, particularly SCLE, but they are also known to induce both SLE and CLE.
What are the recommended immunosuppressive options for patients with antimalarial refractory cutaneous lupus erythematosus (CLE)?
- Systemic Glucocorticoids: IV methylprednisolone for severe cases. Prednisone (20-40 mg/day) with gradual reduction.
- Traditional Immunosuppressives: Azathioprine, Mycophenolate mofetil, Methotrexate.
- Biologics: Anti-TNF medications, B-lymphocyte stimulator inhibitors, Rontalizumab, sifalimumab, Anifrolumab, Tofacitinib, and ruxolitinib.
What is the purpose of fat transplant in lupus patients?
For atrophic scarring and burned out lupus panniculitis.
What should be avoided in cosmetic treatments for lupus patients?
Collagen and foreign cosmetic material injections should be avoided due to potential complications.
What preventive measures should be taken for patients with cutaneous lupus erythematosus (CLE)?
- Physical Protection: Use broad spectrum sunscreens and protective clothing against UV light.
- Cigarette Smoking: Patients are encouraged to discontinue smoking as it is associated with CLE and less so with SLE.
- Awareness of Antimalarial Treatment: Smoking is not responsive to antimalarials, hence cessation is crucial for management.
What conditions should always be ruled out when diagnosing Acute Cutaneous Lupus Erythematosus (ACLE)?
The conditions that should always be ruled out for ACLE include:
- Cancer
- Toxic epidermal necrolysis
- Chronic Cutaneous Lupus Erythematosus (CCLE)
- Tinea imbricata
- Cutaneous T-cell lymphoma
- Lupus panniculitis
- Infectious granulomatous (like fungal/ atypical/mycobacterial organisms)
- Calciphylaxis
What are the first-line therapeutic options for lupus erythematosus-specific skin disease?
The first-line therapeutic options include:
- Topical glucocorticoids: Class I topical steroid daily to twice daily for 2 weeks, alternating with pimecrolimus 1% or tacrolimus 0.1% twice daily for 2 weeks.
- Intralesional triamcinolone acetonide: 2.5-10 mg.
Dosage for topical glucocorticoids is based on the severity of the condition and patient response.
What are the key differential diagnoses for suspected Acute Cutaneous Lupus Erythematosus (ACLE)?
The key differential diagnoses for ACLE include:
- Localized: Acne rosacea, Dermatitis (contact/irritant), Drug-induced hypersensitivity reaction
- Consider: Isomorphic response (Koebner phenomenon), Psoriasis, Erythema multiforme
- Always Rule Out: CLE, Toxic epidermal necrolysis, DLE, Cutaneous T-cell lymphoma, Infectious processes (e.g., fungal, mycobacterial)
What are the first-line therapeutic options for managing skin disease in patients with Lupus Erythematosus?
The first-line therapeutic options for skin disease in Lupus Erythematosus include:
- Topical glucocorticoids: Class I topical steroids daily to twice daily for 2 weeks, alternating with pimecrolimus 1% or tacrolimus 0.1% twice daily for 2 weeks.
- Intralesional triamcinolone acetonide: 2.5-10 mg.
- Hydroxychloroquine: 6.5 mg/kg/day based on ideal body weight.
- Chloroquine: 3.5 mg/kg/day based on ideal body weight.
