Clinical Manifestations of Systemic Lupus Erythematosus.pdf

SLE is a multi system disease. It is important to study the various features of this disease so as to identify the disease early.

This presentation describes the clinical manifestations of SLE.

1. SYSTEMIC LUPUS
ERYTHEMATOSUS
CLINICAL MANIFESTATIONS

2. Musculoskeletal manifestations
Polyarthritis
-characterized by soft tissue swelling and tenderness in joints and/or
tendons
-Most commonly seen in hands , wrists and knees
-Most people with SLE have intermittent polyarthritis
-Joint deformities ( hands and feet ) develop only in 10% - often reducible
-Joint erosions can be identified by ultrasound in 10–50% of patients
Individuals with erosions may fulfill criteria for both RA and SLE (“rhupus”).

3. Jaccoud’s arthropathy ( JA ) is a
deforming, non-erosive arthritis,
occurring in 10–35% of SLE
patients.

While JA can involve all joints, it
most commonly manifests as
severe deformations of the
hands, including ulnar deviation,
swan neck and boutonniere
deformities, and Z-deformity of the
thumb, with multiple non-erosive
subluxations. Ulnar drift with
subluxation of the MCP joint is
often the first sign, whereas swan
neck, boutonniere, and
Z-deformities may occur at later
stages

4. Ischemic necrosis of bone ( INB )
-INB prevalence is increased in SLE, especially in patients treated with
systemic glucocorticoids.
-If pain persists in a single joint, such as knee, shoulder, or hip, a diagnosis
of INB should be considered, particularly if there are no other
manifestations of active SLE.
Myositis / Myalgias
➔Most patients have myalgias without frank myositis.
➔Myositis with clinical muscle weakness, elevated creatine kinase levels,
positive magnetic resonance imaging (MRI) scan, and muscle necrosis and
inflammation on biopsy can occur
➔Glucocorticoid therapies (commonly) and antimalarial therapies (rarely)
can cause muscle weakness; these adverse effects must be distinguished
from active inflammatory disease.

5. Cutaneous manifestations
Discoid lupus erythematosus (DLE)
●The most common chronic dermatitis in
lupus
●Lesions are roughly circular with slightly
raised, scaly, hyperpigmented erythematous
rims and depigmented, atrophic centers in
which all dermal appendages are
permanently destroyed.
●Lesions can be disfiguring, particularly on the
face and scalp.
●Only 5% of people with DLE have SLE
(although half have positive ANA); however,
among individuals with SLE, as many as 20%
have DLE.

6. Acute SLE rash
●The most common acute SLE rash
is a photosensitive, slightly raised,
occasionally scaly erythema on the
face (particularly the cheeks and
nose—the “butterfly” rash), ears,
chin, V region of the neck and
chest, upper back, and extensor
surfaces of the arms.
●Worsening of this rash often
accompanies flare of systemic
disease.

7. Subacute cutaneous lupus
erythematosus (SCLE)
●consists of scaly red patches,
similar to psoriasis, or circular,
flat, red-rimmed (“annular”)
lesions.
●Patients with these
manifestations are exquisitely
photosensitive; most have
antibodies to Ro (SS-A).

8. Other SLE rashes include
recurring urticaria, lichen
planus– like dermatitis,
bullae, and panniculitis
(“lupus profundus”).
Rashes can be minor or
severe; they may be the
major disease
manifestation.

9. Small ulcerations on the oral or nasal
mucosa are common in SLE; the
lesions resemble aphthous ulcers
and may or may not be painful.

10. RENAL MANIFESTATIONS
Nephritis is usually the most serious manifestation of SLE, particularly
because nephritis and infection are the leading causes of mortality in the first
decade of disease.
Because nephritis is asymptomatic in most lupus patients, urinalysis
should be ordered in any person suspected of having SLE.
The classification of lupus nephritis is primarily histologic ( ISN / RPS
classification ).
Renal biopsy is recommended for every SLE patient with any clinical evidence
of nephritis; results are used to plan current therapies and their duration.

13. Patients with dangerous proliferative forms of glomerular damage (ISN III
and IV) usually have microscopic hematuria and proteinuria (>500 mg per 24
h); approximately one-half develop nephrotic syndrome, and most develop
hypertension.
Overall, in the United States, ~20% of individuals with lupus diffuse
proliferative glomerulonephritis (DPGN) die or develop ESRD within 10 years
of diagnosis.
Such individuals require aggressive control of SLE and of the
complications of renal disease and of therapy unless damage is
irreversible.

14. African Americans, Hispanics, and Asians/Pacific Islanders are more likely to
develop nephritis than Caucasians.
African Americans are more likely to develop ESRD than are whites, even with
the most current therapies.
Approximately 20% of SLE patients with proteinuria (usually nephrotic) have
membranous glomerular changes without proliferative changes on renal
biopsy. Their outcome is better than for those with DPGN, but patients with
class V and nephrotic range proteinuria should be treated in the same way as
those with classes III or IV proliferative disease.

15. Lupus nephritis is usually an ongoing disease, with flares requiring
re-treatment or increased treatment over many years.
For most people with lupus nephritis, accelerated atherosclerosis becomes
important after several years of disease; attention must be given to control of
systemic inflammation, blood pressure, hyperlipidemia, and hyperglycemia.

16. NERVOUS SYSTEM MANIFESTATIONS
●Are the symptoms a result
from SLE or another condition
(such as infection in
immunosuppressed individuals
or side effects of therapies) ?

If symptoms are related to SLE,
●Are they caused by a diffuse
process (requiring
immunosuppression) or
vascular occlusive disease
(requiring anticoagulation) ?



The major neurologic
manifestations include
●Cognitive dysfunction
●Headache
●Seizures
●Psychosis
●Myelopathy

17. The most common manifestation of diffuse CNS lupus is cognitive
dysfunction, including difficulties with memory and reasoning.
Headaches are also common. When excruciating, they often indicate
SLE flare; when milder, they are difficult to distinguish from migraine
or tension headaches.
Seizures of any type may be caused by lupus; treatment often requires
both antiseizure and immunosuppressive therapies.

18. Psychosis can be the dominant manifestation of SLE; it must be
distinguished from glucocorticoid-induced psychosis.
Glucocorticoid induced psychosis usually occurs in the first weeks of
glucocorticoid therapy, at daily doses of ≥40 mg of prednisone or
equivalent; psychosis resolves over several days after glucocorticoids
are decreased or stopped.
Myelopathy is often disabling; rapid initiation of immunosuppressive
therapy including high-dose glucocorticoids is standard of care.

19. VASCULAR OCCLUSIONS
The prevalence of transient ischemic attacks, strokes, and myocardial
infarctions is increased in patients with SLE.

These vascular events are increased in, but not exclusive to, SLE patients
with antibodies to phospholipids (antiphospholipid antibodies).
Ischemia in the brain can be caused by focal occlusion (either
noninflammatory or associated with vasculitis) or by embolization from
carotid artery plaque or from vegetations of Libman-Sacks endocarditis.

20. Appropriate tests for antiphospholipid antibodies and for sources of
emboli should be ordered in such patients to estimate the need for, intensity
of, and duration of anti-inflammatory and/or anticoagulant therapies.
When it is most likely that a cerebral event results from clotting, long-term
anticoagulation is the therapy of choice.
Two processes can occur at once—vasculitis plus bland vascular
occlusions—in which case it is appropriate to treat with anticoagulation plus
immunosuppression.

21. In SLE, myocardial infarctions are primarily manifestations of
accelerated atherosclerosis.
The increased risk for vascular events is three to tenfold overall and is highest
in women aged <49 years compared to age-matched controls.
Characteristics associated with increased risk for atherosclerosis include male
gender, older age, hypertension, dyslipidemia, diabetes, dysfunctional
proinflammatory high-density lipoproteins, high disease activity, high
glucocorticoid dose, and high serum homocysteine and leptin.
Statin therapies reduce levels of low-density lipoproteins (LDL) in SLE patients;
significant reduction of all-cause mortality by statins has been shown in SLE
patients with renal transplants and in an epidemiologic study of a large
number of patients in Taiwan.

22. PULMONARY MANIFESTATIONS
The most common pulmonary manifestation of SLE is pleuritis
with or without pleural effusion. This manifestation, when mild, may
respond to treatment with nonsteroidal anti-inflammatory drugs
(NSAIDs); when more severe, patients require a brief course of
glucocorticoid therapy.
Pulmonary arterial hypertension occurs in a small proportion of SLE
patients and should be treated in the same way as idiopathic
pulmonary hypertension.

23. Pulmonary infiltrates also occur as a manifestation of active SLE and are
difficult to distinguish from infection on imaging studies.
Life-threatening pulmonary manifestations include
●interstitial inflammation leading to fibrosis (histologic pattern mimics
usual diffuse interstitial pneumonitis),
●shrinking lung syndrome, and
●intra-alveolar hemorrhage;
all of these probably require early aggressive immunosuppressive therapy as
well as supportive care.

24. Shrinking lung syndrome (SLS) is a rare complication of systemic
autoimmune disease. It occurs most commonly in systemic lupus
erythematosus but there are rare reports of it developing in systemic
sclerosis, Sjogren's syndrome, and rheumatoid arthritis.

It should be suspected in a patient with a relevant autoimmune disease
who presents with exertional shortness of breath with or without pleuritic
chest pain with radiographic evidence of unilateral or bilateral elevation of
the hemidiaphragm or diaphragms.

Reduced lung volumes and a restrictive deficit on lung function testing are
typical. It is a diagnosis of exclusion and therefore other investigations such
as computed tomography of the chest are undertaken to exclude
competing diagnoses

25. CARDIAC MANIFESTATIONS
Pericarditis is the most frequent cardiac
manifestation; it usually responds to
anti-inflammatory therapy and infrequently
leads to tamponade.
More serious cardiac manifestations are
myocarditis and fibrinous endocarditis of
Libman-Sacks.
Myocardial inflammation can be associated
with left ventricular dysfunction and heart
failure.
Libman-Sacks
endocarditis (LSE) has
small- or medium-sized
vegetations on either or
both sides of the valve
leaflets.

26. Overall, lupus patients have a 2.7-fold higher risk of developing heart
failure compared with the general population. Arrhythmias are
frequent. Endocardial involvement can lead to valvular insufficiencies,
most commonly of the mitral or aortic valves, or to embolic events.
Patients with SLE are at increased risk for myocardial infarction,
usually due to accelerated atherosclerosis, which probably results
from immune attack, chronic inflammation, and/or chronic oxidative
damage to arteries.

27. It has not been proven that glucocorticoid or other
immunosuppressive therapies lead to improvement of lupus
myocarditis or endocarditis, but it is usual practice to administer a trial
of high-dose steroids along with appropriate supportive therapy for
heart failure, arrhythmia, or embolic events

28. HEMATOLOGIC MANIFESTATIONS
The most frequent hematologic manifestation of SLE is anemia, usually
normochromic normocytic, reflecting chronic illness and consequent impaired
utilization of iron.
Hemolysis can be rapid in onset and severe, requiring high-dose
glucocorticoid therapy.
Leukopenia is also common and almost always consists of lymphopenia, not
granulocytopenia; lymphopenia rarely predisposes to infections and by itself
usually does not require therapy.

29. Thrombocytopenia may be a recurring problem.
If platelet counts are >40,000/μL and abnormal bleeding is absent, therapy
may not be required.
High-dose glucocorticoid therapy (e.g., 1 mg/kg per day of prednisone or
equivalent) is usually effective for the first few episodes of severe
thrombocytopenia.
Recurring or prolonged hemolytic anemia or thrombocytopenia, or disease
requiring an unacceptably high dose of daily glucocorticoids, should be
treated with additional strategies such as rituximab, platelet growth factors,
and/or splenectomy.

30. GASTROINTESTINAL MANIFESTATIONS
Nausea, sometimes with vomiting, and diarrhea can be manifestations of an SLE
flare.
Diffuse abdominal pain can be caused by autoimmune peritonitis and/or
intestinal vasculitis.
Increases in serum aspartate aminotransferase (AST) and alanine aminotransferase
(ALT) are common when SLE is active. These manifestations usually improve
promptly during systemic glucocorticoid therapy.
Vasculitis involving the intestine can be life-threatening; perforations, ischemia,
bleeding, and sepsis are frequent complications. Aggressive immunosuppressive
therapy with high-dose glucocorticoids is recommended for short term control;
evidence of recurrence is an indication for additional immunosuppression.

31. OCULAR MANIFESTATIONS
Sicca syndrome (Sjögren’s syndrome )and nonspecific conjunctivitis
are common in SLE and rarely threaten vision. In contrast, retinal
vasculitis and optic neuritis are serious manifestations: blindness can
develop over days to weeks.
Aggressive immunosuppression is recommended, although there are
no controlled trials to prove effectiveness.
Complications of systemic and intraorbital glucocorticoid therapy
include cataracts (common) and glaucoma.

35. REFERENCE