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It may be definite or incomplete
SLE.
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It may be a connective tissue disease
that has clinical overlap with SLE (i.e. Sjögren's
syndrome).
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It may be an unspecified connective
tissue disease.
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And, in some instances, there will
not be any rheumatic disease at all.
Individuals with incomplete lupus erythematosus
(ILE) are those who have some clinical features of
SLE, but not all of the required diagnostic criteria
based on the classification devised by the American
College of Rheumatology. According to this classification,
a person is said to have SLE if at least 4 of the
11 established criteria are met (see Table
1). Someone who meets only a few of these
criteria might be considered to have ILE by their
rheumatologist.
Common features
of incomplete lupus
Common features of ILE are:
- tiredness
- low-grade fever
- joint pain
- hair loss
- the facial "butterfly rash"
- a positive test for antinuclear antibodies, or ANA.
This individual probably does have SLE,
but shows only two ACR classification criteria: the
butterfly rash and a positive ANA.
Only a few studies have been published
on the clinical presentation and outcome of ILE cases,
and the variation in selection criteria and methods
used in these studies makes it difficult to reach
conclusions.
Nevertheless, people with ILE appear
to have a mild disease, frequently with arthritis,
cutaneous involvement, and a positive ANA test. Organ-threatening
disease such as kidney or brain involvement seems
to be uncommon.
Can incomplete
lupus develop into SLE?
The immediate question asked by people who are diagnosed
with ILE is whether the disease will stay the same
or will evolve into SLE.
Is there any way to predict who will
develop SLE? A study that attempted to answer these
questions was performed at the Universidad Central
del Caribe School of Medicine, Bayamón, Puerto
Rico. The results of this study were published in
Lupus 2000: 9, 110-115.
Because of the great clinical overlap
that exists between SLE and other connective tissue
diseases, the selection criteria were chosen very
carefully (see Table 2).
Excluded individuals were those who fulfilled the
classification criteria for other rheumatic diseases,
or who presented distinctive clinical features of
other connective tissue diseases-for example, individuals
with fibromyalgia syndrome, which shares many symptoms
with SLE.
ILE study results
In this study, 87 Puerto Ricans (82 women, 5 men)
met the study criteria for ILE, with an average disease
duration of 4.4 years. These individuals were followed
for an average of 2.2 years. The participants whose
diagnosis remained ILE throughout the study were then
compared to two different groups: those in the study
whose disease evolved into SLE, and members of a well-established
group of 94 SLE patients.
Of the total of 87 study subjects who
were initially diagnosed with ILE, only eight cases
(9 percent, all female) evolved into SLE. The average
age at onset was lower in those who developed SLE
(24 years) than in those who remained with ILE (34
years).
The average time interval between onset
of ILE and development of SLE was 4.4 years. None
of the individuals whose disease evolved into SLE
had neurologic, cardiac, or pulmonary involvement.
Only one person developed kidney disease, but never
developed renal insufficiency or severe renal damage.
At baseline, individuals who remained
with ILE were less likely than those whose disease
evolved into SLE to have:
- photosensitivity
- elevated anti double-stranded DNA (anti-dsDNA) levels,
or
- low C3 complement levels.
And when the initial symptoms of ILE
were compared with all cases of SLE, those with ILE
were less likely to have:
- photosensitivity
- butterfly rash
- oral ulcers
- Raynaud's phenomenon
- arthritis
- serologic abnormalities (such as low serum complements
C3 and C4, elevated anti-dsDNA, positive anti-Sm,
anti-RNP, anti-Ro and anti-La antibodies).
Statistical analysis showed that the most important
factors to predict the evolution of ILE into SLE were:
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butterfly rash
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oral ulcers
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elevated anti-dsDNA, and
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low C4.
Conclusions
The findings of our study are in agreement with other
studies in three ways.
1) First, persons with ILE seem to have mild disease
in which life-threatening complications are rare.
2) Second, only a small number of those with ILE develop
further SLE manifestations.
3) Finally, those who develop SLE still continue with
a mild disease.
On the other hand, marked differences
are found in the predictors of disease evolution among
ILE studies. Other studies have identified discoid
(skin) lupus, homogeneous pattern of ANA, and positive
anti-Sm antibodies as risk factors for SLE occurrence.
In addition to different selection criteria, these
variations could be attributed to ethnic or racial
influence. It is known that clinical manifestations,
immunologic features, and outcome of SLE may vary
substantially among people from different ethnic origins.
Further studies are needed to determine
the outcome of ILE and to identify the risk factors
of disease progression. This information will help
to provide better medical care to people with ILE,
and to identify those who may develop disease progression.
To achieve these goals it will be necessary
to:
1) establish more definitive and uniform criteria,
2) follow patients for longer periods of time, and
3) study patients of different ethnic backgrounds.
About the Author
Dr. Luis M. Vilá is Chief and Program Director,
Division of Rheumatology, at the University of Puerto
Rico School of Medicine in San Juan, PR. He was previously
an Assistant Professor, Division of Rheumatology,
at Universidad Central del Caribe School of Medicine
in Bayamón, PR. Dr. Vilá is one of the
Investigators for the LUMINA Study: "Lupus in
minority populations: Nature vs Nurture."
Table 1. The
11 Criteria Used for the Diagnosis of Lupus Erythematosus
| Criterion |
Definition
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| Malar Rash |
Rash over the cheeks |
| Discoid
Rash |
Red raised patches |
| Photosensitivity |
Reaction to sunlight, resulting
in the development of or increase in skin rash |
| Oral Ulcers |
Ulcers in the nose or mouth, usually
painless |
| Arthritis |
Nonerosive arthritis involving two
or more peripheral joints (arthritis in which
the bones around the joints do not become destroyed) |
| Serositis |
Pleuritis or pericarditis (inflammation
of the lining of the lung or heart) |
| Renal Disorder |
Excessive protein in the urine (greater
than 0.5 gm/day or 3+ on test sticks) and/or cellular
casts (abnormal elements the urine, derived from
red and/or white cells and/or kidney tubule cells) |
Neurologic
Disorder |
Seizures (convulsions) and/or psychosis
in the absence of drugs or metabolic disturbances
which are known to cause such effects |
Hematologic
Disorder |
Hemolytic anemia or leukopenia (white
blood count below 4,000 cells per cubic millimeter)
or lymphopenia (less than 1,500 lymphocytes per
cubic millimeter) or thrombocytopenia (less than
100,000 platelets per cubic millimeter). The leukopenia
and lymphopenia must be detected on two or more
occasions. The thrombocytopenia must be detected
in the absence of drugs known to induce it. |
Antinuclear
Antibody |
Positive test for antinuclear antibodies
(ANA) in the absence of drugs known to induce
it. |
Immunologic
Disorder |
Positive anti-double stranded anti-DNA
test, positive anti-Sm test, positive antiphospholipid
antibody such as anticardiolipin, or false positive
syphilis test (VDRL). |
Adapted from: Tan, E.M., et. al.
The 1982 Revised Criteria for the Classification of
SLE. Arthritis & Rheumatism 25:1271-1277.
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Table 2: Selection
Criteria For Incomplete Lupus Erythematosus
Inclusion criteria:
1. Clinically-apparent lupus, having
at least one but less than four of the ACR criteria
for the classification of SLE.
Exclusion criteria:
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Drug-induced lupus
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Fulfilling classification criteria
for the diagnosis of the following rheumatic diseases:
rheumatoid arthritis, systemic sclerosis, polymyositis,
dermatomyositis, Sjögren's syndrome, vasculitides
(temporal arteritis, Takayasu arteritis, polyarteritis
nodosa, Wegener's granulomatosis, Churg-Strauss
syndrome, Henoch-Schönlein purpura, hypersensitivity
vasculitis) Behçet's disease, antiphospholipid
syndrome, seronegative spondyloarthropathies (ankylosing
spondylitis, psoriatic arthritis, Reiter's syndrome)
or fibromyalgia syndrome (FMS).
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Presence of any of the following
connective tissue disease features: erosive arthritis,
scleroderma clinical signs (skin tightening, calcinosis,
telangiectasia), polymyositis features (proximal
muscle weakness, elevation of muscle enzymes), dermatomyositis
rashes (heliotrope rash, Gottron's rash), sicca
symptoms (dry eyes, dry mouth), sacroiliitis, sausage
digits, or FMS tender points.
> back to the top
July 30, 2003
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