Acute Cutaneous Lupus Erythematosus

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ACLE manifestations indicate SLE. Acute skin lesions include "butterfly" rash, generalized erythema, and bullous lesions. Butterfly rash is the most common acute skin lesion of SLE, developing as an erythematous, sometimes raised, lesion most often in a malar distribution. It lasts from days to weeks and often is painful or pruritic. A temporal association with sun exposure is common.

ACLE is reported to occur in about 50%-60% of patients at the onset of SLE, and during follow-up, the frequency of skin involvement reaches about 80% (Table 13.1). Butterfly rash is present in less than 50% of patients at disease onset, a reminder not to exclude SLE when this diagnostic clue is missing. Because no other symptom is 100% specific for SLE and sensitivity varies for the different manifestations, classification of SLE is made using classification criteria (Table 13.2). The specificity of the cutaneous scoring items is very high (96%-99%), and the sensitivity ranges from 18% for discoid lesions to 57% for butterfly rash (photosensitivity, 43%; oral ulcers, 27%) (Tan et al. 1982).

The prevalence of SLE is higher than that of CCLE, and it varies from 12 cases per 100,000 population in Britain to 39 cases per 100,000 population in Sweden. Although African-Americans have a high prevalence of this disease, SLE is exceedingly rare among blacks living in Africa (Hart et al. 1983). The disease occurs primarily in young women and ranges in severity from a mild disease to a devastating illness with multiorgan involvement (Gladman and Urowitz 1998).

Indicators of SLE are constitutional symptoms, which mostly occur in patients during the course of active disease and often are the earliest symptoms. Fatigue, fever,

Table 13.1. Frequency of lupus manifestations8

Manifestation

Number of

Number of

Number of

patients (%)

patients (%)

patients (%)

[n=108]

[n=605]

[n=520]

Arthralgia

77

85

92

Constitutional

73

84

86

Skin

57

81

72

Arthritis

56

63

92

Renal

44

77

46

Raynaud's phenomenon

33

58

18

Lymphadenopathy

25

32

59

Central nervous system

24

54

26

Pleurisy

23

37

45

Pericarditis

2G

29

31

Mucous membranes

18

54

9

Vasculitis

10

37

21

Lung

9

17

0

Myositis

7

5

0

Nephrotic syndrome

5

11

23

Myocarditis

1

4

8

a The frequency of lupus manifestations at onset is based on 108 patients and at any time for 605 patients (before 1990) and 520 patients (after 1990) (Gladman and Urowitz 1998).

a The frequency of lupus manifestations at onset is based on 108 patients and at any time for 605 patients (before 1990) and 520 patients (after 1990) (Gladman and Urowitz 1998).

Table 13.2. The 1982 revised American College of Rheumatology (ACR) criteria for classification of systemic lupus erythematosusa

Criterion

Definition

1.

Malar rash

Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds

2.

Discoid rash

Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions

3.

Photosensitivity

Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation

4.

Oral ulcers

Oral or nasopharyngeal ulceration, usually painless, observed by a physician

5.

Arthritis

Nonerosive arthritis involving 2 peripheral joints, characterized by tenderness, swelling, or effusion

6.

Serositis

Pleuritis - convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion Pericarditis - documented by electrocardiographic or rub or evidence of pericardial effusion

7.

Renal disorder

Persistent proteinuria - >0.5 g/d or >3+ if quantitation is not performed

Cellular casts - may be red blood cells, hemoglobin, granular, tubular, or mixed

8.

Neurologic

Seizures - in the absence of offending drugs or known metabolic

disorder

derangements, e. g., uremia, ketoacidosis, or electrolyte imbalance Psychosis - in the absence of offending drugs or known metabolic derangements, e. g., uremia, ketoacidosis, or electrolyte imbalance

9.

Hematologic

Hemolytic anemia - with reticulocytosis

disorder

Leukopenia - <4,000/mm total on 2 occasions Lymphopenia - <1,500/mm on 2 occasions Thrombocytopenia - <100,000/mm in the absence of offending drugs

10. Immunologic

Anti-DNA - antibody to native DNA in abnormal titer

disorder

Anti-Sm - presence of antibody to Sm nuclear antigen Antiphospholipid antibodies (positive reaction at 2 time points with at least 6 weeks' separation):

- Increased IgG or highly increased IgM anti-cardiolipin antibodies or

- Positive lupus anticoagulants using a standard method or

- Positive serologic test results for syphilis

11.

Antinuclear

An abnormal titer of antinuclear antibody by immunofluores-

antibody

cence or an equivalent assay at any point in time and in the absence of drugs known to be associated with "drug-induced lupus" syndrome

a The proposed classification is based on 11 criteria. For the purpose of identifying patients in clinical studies, a person shall be said to have systemic lupus erythematosus if any four or more of the 11 criteria are present, serially or simultaneously, during any interval of observation (Tan et al. 1982).

arthralgia and myalgia, and weight changes are the most common of these complaints (Hildebrand and Muller 2002).

Fever due to active disease, infection (favored by immunosuppression), and drug use is another common and nonspecific symptom that occurs in more than 50% of patients with SLE.

Musculoskeletal Symptoms

Arthralgia, myalgia, and arthritis represent the most common presenting complaints in SLE. Arthritis tends to be migratory and asymmetrical. It can affect any joint, but the small joints of the hands, wrists, and knees are involved most frequently. Arthritis classically is nonerosive and usually nondeforming. Soft-tissue swelling is common, and effusions, when they occur, are usually mildly inflammatory. However, as many as 10% of patients develop hand deformities due to chronic arthritis and tendinitis, called "Jaccoud arthropathy", which mimic the changes observed in rheumatoid arthritis but lack erosions.

Renal Involvement

The kidney is the most commonly involved visceral organ in SLE, although involvement is asymptomatic in its earliest stages. Although only approximately 50% of patients develop clinically evident renal disease, results of kidney biopsy demonstrate some degree of renal involvement in almost all patients with SLE. Nephritis usually develops within the first few years after onset of disease. Clinically, patients may present with an acute nephritic picture, nephrotic syndrome, acute renal failure, or chronic renal insufficiency. Rarely, acute renal pain can be present secondary to an associated pyelonephritis or to vasculitis with renal infarction. Signs of nephropathy was the variable with the highest statistical relevance for distinguishing between patients with CLE (DLE/SCLE) and SLE in the European Centres of Dermatology (Tebbe et al. 1997).

Central Nervous System Symptoms

Neuropsychiatric symptoms are common manifestations of SLE. The multiple central nervous system manifestations can be divided into inflammatory and thrombotic processes, the latter most commonly presenting as thrombotic or thromboembolic stroke and often associated with the presence of antiphospholipid antibodies. Other central nervous system manifestations, some of which are related to a vasculitic lupus cerebritis, include headache, seizures (focal or generalized), organic brain syndrome, and psychosis.

A high percentage of patients with SLE have evidence of faint cognitive impairment by neurocognitive testing, but whether this represents true encephalopathy, a feature of previous neurologic damage, medication-induced effects, some other process, or some combination of the above is unclear.

Pulmonary Involvement

Pulmonary manifestations of SLE include acute and chronic processes. Pleurisy with pleuritic chest pain with or without pleural effusions is the most common feature of acute pulmonary involvement. Acute lupus pneumonitis and chronic lupus interstitial disease are unusual causes of shortness of breath in SLE. Other conditions, especially infection, should be considered. Diffuse alveolar hemorrhage is an acute, life-threatening pulmonary process in SLE, often occurring with other evidence of active lupus.

Cardiac Manifestations

Pericarditis (serositis), verrucous endocarditis (Libman-Sacks), myocarditis, and accelerated development of coronary artery disease occur at higher rates in patients with SLE. The most common cardiac manifestation is chest pain due to pericarditis, with or without pericardial effusion. Whereas atherosclerotic coronary artery disease is associated with inactive SLE and is thought to be in part secondary to chronic steroid therapy, pericarditis and myocarditis occur in patients with active SLE. Coronary vasculitis occurs only very rarely, and coronary thrombosis related to antiphos-pholipid antibodies also is a rare event.

Prognosis

An improvement in the survival rate has occurred during the past few years: the 10-year survival rate now approaches 90%, compared with a 5-year survival rate of less than 50% in the 1950s (Urowitz and Gladman 2000). The decrease in mortality can be attributed to facilitated diagnosis by feasibility of serologic markers, leading to identification of milder forms and cases at earlier stages and allowing for swift disease-specific treatment.

Death due to complications of SLE itself, such as nephritis, occurs at a higher rate within 5 years of onset of symptoms. Infectious complications coincident with active SLE and its treatment with immunosuppressives, cardiovascular complications, and malignancy are now the most common causes of death in patients with SLE and chronic damage due to long-lasting disease and therapy (Manger et al. 2002, Urowitz and Gladman 2000). This is explained by a variety of factors, including endothelial cell injury during active SLE, premature atherosclerosis, and dyslipidemia related to renal disease or long-term administration of corticosteroids (Urowitz and Gladman 2000a, Urowitz et al. 2000). Despite the improvement in the overall survival rate,patients with SLE still have a death rate three times higher than that of the general population, and patients with isolated skin and musculoskeletal involvement have higher survival rates than those with renal and CNS disease (Gladman and Urowitz 1998).

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