Ass. Sklyannaya E.V.
Systemic lupus erythematosus (SLE) is an
autoimmune disease in which organs, tissues,
and cells undergo damage mediated by tissue-
binding autoantibodies and immune
Ninety percent of patients are women of child-
Prevalence of SLE is 15 to 50 per 100,000.
hypersensitivity of T and B lymphocytes
production of pathogenic autoantibodies
formation of immune complexes
sequestration and destruction of Ig-coated
fixation and cleaving of complement proteins
release of chemotaxins, vasoactive peptides,
and destructive enzymes into tissues
In skin - deposition of Ig at the dermal-epidermal junction (DEJ),
injury to basal keratinocytes, and inflammation dominated by T
lymphocytes in the DEJ and around blood vessels and dermal
In renal biopsies - proliferative changes in mesangium and in
glomeruli, predominantly membranous changes to scarred
In blood vessels - leukocytoclastic vasculitis
In lymph node biopsies - nonspecific diffuse chronic
Systemic Manifestations (fatigue, myalgias,
arthralgias, fever, prostration, weight loss)
Musculoskeletal Manifestations (polyarthritis,
Renal Manifestations (Nephritis)
Nervous System Manifestations (cognitive
dysfunction, headaches, seizures, psychosis,
(lupus dermatitis: discoid
systemic rash, subacute
The classic malar rash, also known as
a butterfly rash, with distribution over
the cheeks and nasal bridge. Note that
the fixed erythema, sometimes with
mild induration as seen here,
characteristically spares the nasolabial
folds. Photosensitive SLE rashes
typically occur on the face or
extremities, which are sun-exposed
Tests for Autoantibodies Diagnostically, the most
important autoantibodies to detect are ANA since the
test is positive in >95% of patients, usually at the
onset of symptoms. High-titer IgG antibodies to
double-stranded DNA (dsDNA) (but not to single-
stranded DNA) are specific for SLE.
Standard Tests for Diagnosis Screening tests for
complete blood count, platelet count, and urinalysis
may detect abnormalities that contribute to the
diagnosis and influence management decisions.
1. Malar rash.
2. Discoid rash.
4. Oral ulcers.
7. Renal disorder.
8. Neurologic disorder.
9. Hematologic disorder.
10. Immunologic disorder.
11. Antinuclear antibodies.
Antiphospholipid Syndrome, Fibromyalgia,
Hepatitis C, Infectious Mononucleosis, Infective
Endocarditis, Lyme Disease, B-Cell Lymphoma,
Mixed Connective-Tissue Disease, Polyarteritis
Nodosa, Preeclampsia (Toxemia of Pregnancy),
Rheumatic Fever, Rheumatoid Arthritis,
Scleroderma, Serum Sickness, Thrombotic
Thrombocytopenic Purpura, Undifferentiated
Connective-Tissue Disease, Drug-induced lupus
erythematosus, Vasculitis, Leukemia, Neoplasia,
HIV, Multiple sclerosis, Parvovirus or other viral
Survival in patients with SLE is 90 to 95%
at 2 years, 82 to 90% at 5 years, 71 to 80% at
10 years, and 63 to 75% at 20 years.
Systemic sclerosis (SSc) is a chronic
multisystem disorder of unknown etiology
characterized clinically by thickening of the
skin caused by accumulation of connective
tissue and by structural and functional
abnormalities of visceral organs, including the
gastrointestinal tract, lungs, heart, and kidneys.
The estimated incidence of systemic
sclerosis is 19 cases per million population,
and the prevalence of systemic sclerosis has
been estimated at 240 cases per million
population, although reported prevalence has
ranged from 138 to 286 cases per million
population. The risk of systemic sclerosis is 3-
9 times higher in women than in men. The
peak onset occurs in individuals aged 30-50
1. Genetic abnormalities.
2. Infectious agents.
3. Environmental factors.
Systemic sclerosis is
characterized by excessive
fibrosis in the skin and other
affected organs. The skin
and lungs also show
infiltration. A severe
vasculopathy that affects
small arteries and arterioles
is universally present in
affected organs. Platelet
microthrombi are often
found in the lumen of the
1. Systemic sclerosis:
Limited cutaneous disease
Diffuse cutaneous disease
Undifferentiated connective tissue disease
2. Localized scleroderma
Linear scleroderma (en coup de sabre)
Tightening of the skin
in the face, with a
facies and paucity of
digital ulceration, loss
of skin creases, joint
contractures, and sparse
hyperpigmentation in a
Pulmonary function testing
Serum N-terminal pro-brain natriuretic peptide
Cardiac rhythm monitoring
Biopsy of skin and lungs
Preliminary criteria of systemic sclerosis by
the American Rheumatism Association:
major criterion sclerodermatous involvement
proximal to the digits
minor criteria: 1)sclerodactyly,
2)digital pitting scars or tissue loss of the volar
pads of the fingertips, and
3)bibasilar pulmonary fibrosis.
Eosinophilia, Eosinophilia-Myalgia Syndrome,
Eosinophilic Fasciitis, Graft Versus Host Disease,
Mycosis Fungoides, Primary Biliary Cirrhosis,
Pulmonary Hypertension, Primary, Reflex
Sympathetic Dystrophy, Scleroderma. Other
Problems to be Considered: Toxic oil syndrome
(adulterated rape seed oil), Porphyria cutanea tarda,
Digital sclerosis of diabetes mellitus, Vibration
disease, Radiation exposure, Intestinal obstruction,
Infiltrative cardiomyopathy, Nephrogenic fibrosing
dermopathy (nephrogenic systemic fibrosis),
Amyloidosis, Scleromyxedema (generalized lichen
myxedematosus), Scleredema diabeticorum
recombinant human relaxin,
calcium channel blockers
Pulmonary hypertension and scleroderma renal crisis
are the most frequent causes of mortality. Survival
averages 12 years from diagnosis and correlates best
with the clinical disease subtype (diffuse cutaneous vs
limited cutaneous) and extent of organ involvement.
The limited cutaneous subset carries a 10-year
survival rate of 71%. The diffuse cutaneous subset
carries a 10-year survival rate of 21%. Pulmonary
hypertension is a major prognostic factor for survival.