Lupus erythematosus is a spectrum autoimmune disease with unique clinical manifestations and distinct types of cellular or humoral autoimmunity that can manifest in a variety of disorders from chronic cutaneous lupus erythematosus (CCLE) with skin-only involvement to acute systemic lupus erythematosus (SLE), which is life-threatening.
General information
The disease is more prevalent in women, with a male:female incidence ratio of approximately 1:7 – 9.
Age of onset: 20 – 40 years is the most common age, but it can occur in young children and the elderly.
Incidence rate: North America incidence rate is about 40/100,000; China’s female incidence rate is about 1/2000, some regions reach 1/250.
Etiology and pathogenesis
1, genetic.
2.Endocrine: estrogen, estrogen receptor, lactogen
3.Infection: virus
4, Immunity: B-cell hyperfunction, T-cell imbalance, abnormal cytokine expression, abnormal lymphocyte apoptosis
5, physical factors: ultraviolet light
6, Drugs.
Subtypes of cutaneous lupus erythematosus
Chronic cutaneous lupus erythematosus (CCLE): accounting for 15%-20% of the incidence, including: discoid lupus erythematosus (DLE), edematous (swollen) lupus erythematosus, hypertrophic lupus erythematosus, and profound lupus erythematosus (lupus lipofuscinosis LEP).
Subacute cutaneous lupus erythematosus (SCLE): 10%-15%, including two specific types: neonatal lupus erythematosus (NLE), and complement deficiency syndrome.
Acute cutaneous lupus erythematosus (ALE): 30%-50%, including herpetic lupus erythematosus (BSLE).
Giliam classification of lupus erythematosus skin lesions coded by
A. Lupus-specific skin lesions:
1. Acute cutaneous lupus erythematosus ACLE: limited ACLE (cheek rash, butterfly erythema), generalized ACLE (maculopapular rash, cheek rash, photosensitive dermatitis)
2. Subacute cutaneous lupus erythematosus SCLE: 1) annular – polycyclic red edematous plaques with slightly elevated margins; 2) papular – scaly erythematous plaques (disseminated discoid lupus erythematosus, subacute disseminated discoid lupus erythematosus, maculopapular photosensitive lupus erythematosus). Lupus-specific lesions.
3. Chronic cutaneous lupus erythematosus CCLE: classic discoid lupus erythematosus (limited, generalized), swollen lupus erythematosus, hypertrophic lupus erythematosus, deep lupus erythematosus, frostbite like lupus, mossy discoid lupus erythematosus (lupus erythematosus/flat moss overlap).
B. Non-specific lesions of lupus erythematosus: manifestations range from necrotizing and urticarial vasculitis to reticular bruising, Raynaud’s phenomenon, dermal mucinosis and lupus erythematosus maculopapular lesions, etc.
Chronic cutaneous lupus erythematosus (CCLE):
1. Discoid lupus erythematosus (DLE): clinical manifestations are: bright red or light red facial rash with slightly elevated edges, attached with gray-white adherent scales, visible enlarged hair follicle opening, and embedded with corneal emboli.
DLE has a slow progression and generally no systemic symptoms, but some patients can progress to SLE.
2. Warty lupus erythematosus (hypertrophic lupus erythematosus HLE): It occurs on the face and extremities, and is a non-pruritic nodule-like damage.
HLE sometimes has histological features of squamous carcinoma and keratoacanthoma, and some of them can be transformed into squamous carcinoma and metastasis.
3. Edematous (swollen) lupus erythematosus (LET).
4. deep in lupus erythematosus (lupus lipofuscinosis LEP): an intermediate type between DLE and SLE, the lesions are deep subcutaneous nodules or plaques with red or light red surface, and the surface is depressed or necrotic or ulcerated after nodule absorption and atrophic scars after healing.
Subacute cutaneous lupus erythematosus (SCLE).
1. Skin lesions: annular-polycyclic lesions; papular-squamous erythema.
2. Other manifestations: photosensitivity, alopecia, Raynaud’s sign, reticulocutaneous cyanosis, perineural capillary dilation.
3. Clinical accompaniment: fever, arthritis or arthralgia, muscle pain, plagiocele, and mild renal damage.
4. Laboratory tests: anemia signs, LE cells (+), ANA (+), positive Ro(SSA) antibody, La(SSB) antibody.
Special types of SCLE.
Neonatal lupus erythematosus (NLE): occurs in newborns within three months as transient lesions (edematous annular erythema or discoid lesions) with or without congenital heart block. antibodies to Ro/ SSA (+) are serologic markers of the disease.
Complement deficiency syndrome: Defects in many complement components can be associated with LE manifestations, especially C2 and C4 defects. Clinical manifestations are:ring-like SCLE lesions, photosensitivity, Ro/SSA antibodies (+).
Acute cutaneous lupus erythematosus (ACLE).
Acute cutaneous lupus erythematosus: the cutaneous manifestations of SLE, including: facial butterfly erythema, generalized erythema with or without edema.
Blistering lesions are a new subtype of ACLE, also known as: Blistering lupus erythematosus (BSLE), which clinically manifests as single or clusters of blisters or macules at the site of exposure.
Clinical manifestations of SLE.
1. Skin and mucous membrane damage: facial butterfly erythema, perineural erythema and distal curved finger (toe) nail spots are characteristic
2. Fever: >80% of patients, more hyperthermia in young patients
3. Bone and joint manifestations: joint pain and swelling, muscle pain, polyarthritis, aseptic osteonecrosis
4. Damage to kidney and other organs and systems
5, abnormalities of laboratory tests (omitted)
SLE skin and mucous membrane damage characteristics
Butterfly erythema: characteristic skin lesion, showing edematous erythema, bright red or purple-red color
Discoid erythema: infiltrative bright red or pale red spots on the face with slightly elevated edges, covered with gray-white adherent scales, with enlarged hair follicle openings and embedded corns
Vasculitis: purpura, petechiae, painful subcutaneous nodules, extremity necrosis, ulcers, Raynaud’s phenomenon, etc.
Frostbite like damage: purple erythema on fingers (toes) and face
Herpetic damage: blisters, blood blisters.
rosacea-like damage
Cutaneous mucinosis
Calcium deposits in the skin
Oral ulcers: painless, differentiated from leukoplakia
Alopecia: sometimes the only early manifestation of SLE
Features of SLE alopecia: diffuse alopecia, lupus hair, patchy alopecia (pseudo-alopecia)
Renal damage in SLE
National Institutes of Health (NIH): Lupus Nephritis (LN) Pathology Active, Chronic Lesion Index
WHO 1995 pathological staging of LN (omitted)
Clinical typing of lupus nephritis (LN).
1, occult type
2.Chronic nephritis type
3.Nephrotic syndrome type
4.Acute progressive nephritis type
5.Progressive renal insufficiency type
SLE laboratory tests.
Anemia: complete blood cells ↓, especially wbc <4000/ml; LE cells (+)
ANA: 1, anti-ds-DNA antibodies; 2, anti-DNP (anti-nuclear protein) antibodies; 3, saline-extractable nuclear antigens (anti-Sm, anti-nRNP).
Anti-anti-cell plasma component antibodies: Ro antibody, La (SSB) antibody positive
Complement: C1, C4, C3, C2 all decreased, especially C3 decreased
Lupus band test (+)
Clinical significance of lupus band test
LBT is highly specific and helps in diagnosis and differentiation
Normal skin LBT (+) is highly suggestive of SLE and helps to differentiate it from DLE and confirm the diagnosis of SLE without skin lesions, especially lupus nephritis without extra-renal symptoms
Ig class and fluorescence intensity in normal skin LBT are associated with disease activity, development of nephropathy and prognosis
Some authors suggest that LBT correlates with SLE disease activity, disappears with remission, and can be used as an indicator of efficacy
SLE disease assessment (omitted)
Lupus activity calculation criteria (LACC)
Systemic lupus erythematosus activity index (SLEDI)
SLE activity Out score assessment method (omitted)
Assessment of the degree of damage to involved organs (omitted)
Assessment of health status of SLE patients (omitted)
Treatment.
Treatment of skin damage (omitted)
Treatment of systemic drugs (omitted)
Autologous hematopoietic stem cell transplantation (HSCT)
1.Peripheral blood HSCT mobilization
2. In vitro purification and enrichment of CD34 cells
3.Pre-treatment program (non-cleared marrow)
4.Intravenous transfusion of thawed and resuscitated hematopoietic stem cells
Rationale of HSCT.
The polygenic abnormality of SLE is manifested in the abnormal genetic level of pluripotent hematopoietic stem cells (P-HSC), from which abnormal lymphocytes are differentiated and abnormal immunity is generated. By destroying the original abnormal immunity and rebuilding a healthy immune system, it makes it possible to cure SLE from the root!
Drug-induced lupus erythematosus DILE: A considerable number of drugs can cause a syndrome similar to SLE, and patients may have typical SLE clinical signs and serological manifestations. Common predisposing drugs: aromatic amines, hydrazines, mercaptans, benzenes.
DILE diagnostic criteria.
1. history of drug application that clearly induces DILE (3 weeks to 2 years).
2, no history of SLE prior to drug application.
3.positive ANA with at least one clinical sign of SLE.
4. rapid improvement and gradual return to normal of ANA and other abnormal serological indicators after discontinuation of the drug.
Lupus crisis.
Lupus encephalopathy: including organic encephalopathy, epilepsy, cerebrovascular accident, transverse myelitis, aseptic meningitis, diffuse encephalitis, etc.
Hematologic damage: lupus thrombocytopenic purpura, lupus hemolytic crisis, complete hemocytopenia, DIC, etc.
Cardiac emergencies: such as pericarditis, myocarditis, myocardial infarction, congestive heart failure, etc.
Pulmonary lesions: such as acute lupus pneumonia, lupus pulmonary hemorrhage
Acute abdomen: mesenteric vasculitis, perforation, hemorrhage, acute pancreatitis
Acute renal failure
Emergency treatment measures for lupus crisis
1.Glucocorticoid shock therapy
2.Cyclophosphamide shock therapy
3.Gammaglobulin shock therapy
4.Plasma replacement