Clinical Presentation and Systemic Involvement

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Although DLE and SCLE can be easily diagnosed using clinical and histologic criteria, it is still difficult to predict which cases will develop systemic involvement and transition to SLE. The criteria established by the American Rheumatism Association (ARA) (Tan et al. 1982) characterize an inhomogeneous group of patients with cutaneous manifestations of LE. A maximum of 20% of all patients with DLE and 30%-50% of all patients with SCLE have four or more positive ARA criteria, whereas only two to three criteria are fulfilled in most cases of CLE (Beutner et al. 1991,

Higuchi et al. 1978, Parodi et al. 2000). In addition, typical skin lesions for differentiating DLE from SCLE are not included in the ARA list. Using that list, correct diagnosis and proper evaluation of the course of CLE is difficult, if not impossible. There is a need, therefore, to establish suitable diagnostic criteria for CLE.

Univariate statistical analysis comparing a wide panel of clinical and serologic data in patients with either SLE (n=464) or CLE (n=67), mainly DLE, showed that the following parameters were significantly more frequent in SLE than in CLE: female predominance, fever, arthralgia/arthritis, pericarditis, hypertension, pleurisy, oral ulcers, Raynaud's phenomenon,severe headaches, nephritis, proteinuria, anemia, leukopenia, positive antinuclear antibody (ANA) titer, high anti-DNA antibody titer, low complement C3, and elevated erythrocyte sedimentation rate (Wallace et al. 1992).

SCLE is believed to be associated with extracutaneous manifestations in a large proportion of patients. A cohort of 79 patients with SCLE and 58 with SLE was studied and compared concerning clinical presentation, histopathologic findings, and immunserologic data. SCLE differed from SLE by cutaneous changes, significantly less frequent kidney involvement (2.5% vs 48%), serositis (4% vs 17%), and arthritis (47% vs 88%), together with the rare presence of anti-double-stranded (ds) DNA antibodies (1.2% vs 34%) and U1RNP antibodies (1.2% vs 17%) (Chlebus et al. 1995).

If patients with SCLE fulfilling four or more ARA criteria were compared with patients with SLE without these LE-specific skin lesions, remarkable differences would be noted. Photosensitivity was more frequent in patients with SCLE (82%) than in patients without these cutaneous lesions (45%), whereas arthritis, Raynaud's phenomenon, pleurisy, central nervous system disorder, renal disease, anemia, hypocomplementemia, and anti-ds-DNA antibodies were significantly more frequent in patients without SCLE (Lopez-Longo et al. 1997).

From the dermatologic point of view, it is necessary to develop criteria for recognizing patients with CLE at risk to develop SLE. For elucidating this subject, several studies were performed. Classification criteria for CLE have been published by the European Academy of Dermatology and Venereology (EADV) to obtain more reliable parameters for classifying these patients (Beutner et al. 1991). EADV criteria include ARA criteria and other parameters that are more suitable for the evaluation of patients with CLE, such as vasculitis of the fingers, muscle weakness, lupus band test, elevated erythrocyte sedimentation rate, and anti-Ro/SSA and anti-La/SSB antibodies. In one study, 207 patients with chronic CLE and SCLE were comparatively classified according to ARA and EADV criteria. ARA criteria showed a sensitivity of 88%, a specificity of 79%, a positive predictive value of 56%, and a negative predictive value of 96%. EADV criteria showed a sensitivity of only 64% but a specificity of 93%, a positive predictive value of 61%, and a negative predictive value of 94% (Parodi and Rebora 1997). These data support the view that ARA criteria are less useful for evaluating patients with CLE and that selection of EADV criteria has to be improved to obtain a greater rate of sensitivity.

In a prospective multicenter study of 296 patients with LE performed in five cooperating dermatology departments in Germany and Austria, many variables were collected for each patient (Tebbe et al. 1997). The selected variables were most relevant for disease progression. Univariate and multivariate analysis of the data showed that mild signs of nephropathy (proteinuria and hematuria) had the highest statistical relevance for distinguishing patients with CLE (DLE/SCLE) from those with SLE

Table 14.2. Criteria for differentiation between cutaneous and systemic lupus erythematosus (LE)

CLE (n=464)

SCLE (n=79)

DLE/SCLE (n=245)

vs SLEa (n=67)

vs SLEb (n=58)

vs SLEc (n=51)

Clinical symptoms

Oral ulcers

Arthritis

Mild signs of

and immunserologic

Raynaud's

Kidney

nephropathy (pro-

findings more fre-

phenomenon

involvement

teinuria, hematuria)

quently occurring

Fever

Serositis

Arthralgias

in SLE than in

Arthralgia/arthritis

Anti-ds-DNA

ANA titer (>1:320)

CLE

Nephritis, proteinuria

antibodies

ESR elevation

Hypertension

U1RNP anti

Headaches

bodies

Pericarditis

Anemia, leukopenia ESR elevation ANA, anti-ds-DNA antibodies Low complement C3

Pleurisy

Anemia, leukopenia ESR elevation ANA, anti-ds-DNA antibodies Low complement C3

a Wallace et al. 1992,b Chlebus et al. 1998,c Tebbe et al. 1997.

(odds ratio [OR], 4.21; confidence interval [CI], 1.88-9.38), followed by high ANA titer (>1:320) (OR, 3.11; CI, 1.49-6.48), the presence of arthralgias (OR, 3.58; CI, 1.49-8.60), and erythrocyte sedimentation rate elevation (OR, 2.57; CI, 0.97-6.78). In contrast, low ANA titers as well as anti-ds-DNA antibodies had little or no statistical relevance for differentiation (Tebbe et al. 1997). Thus, patients with CLE and signs of nephropathy, arthralgias, and elevated ANA titers (>1:320) should be carefully monitored because they may be at risk for transition to SLE. The individual risk of developing a severe course of the disease increases with the number of positive variables, as calculated by multiplication of the ORs (Table 14.2).

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