Specific Cutaneous Lesions in Patients With Neurosarcoidosis. / Lesiones cutáneas específicas en pacientes con neurosarcoidosis.

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis.

Endpoints for clinical trials of sarcoidosis.

Over the past few years an increasing number of prospective controlled sarcoidosis treatment trials have been completed. Unfortunately, these studies utilize different endpoints making comparisons between studies difficult. At the recent World Association of Sarcoidosis and other Granulomatous disease (WASOG) meeting, a session was dedicated to the evaluation of clinical endpoints for various disease manifestations. These included pulmonary, pulmonary hypertension, fatigue, cutaneous, and a classification of clinical disease phenotypes. Based on the available literature and our current understanding of the disease, recommendations for clinical evaluation were proposed for each disease category. For example, it was recommended that pulmonary studies should include changes in the forced vital capacity. Additionally, it was recommended that all trials should incorporate measurement of quality of life.

Specific cutaneous lesions in patients with systemic sarcoidosis: relationship to severity and chronicity of disease.

BACKGROUND: Specific (granulomatous) cutaneous lesions are seen in 9-37% of cases of systemic sarcoidosis, and are usually classified into maculopapules, plaques, lupus pernio (LP), scar sarcoidosis, and subcutaneous sarcoidosis. Their prognostic significance has not been fully established. AIM: To analyse the relationship between the clinical type of granulomatous cutaneous lesions and the systemic features and prognosis of systemic sarcoidosis. METHODS: The clinical charts of 86 patients (19 men, 67 women, mean age 46.82 years) with systemic sarcoidosis and granulomatous cutaneous involvement followed up for > 2 years at Bellvitge University Hospital were reviewed. RESULTS: Cutaneous lesions developed before or at the time of diagnosis of systemic sarcoidosis in 80.23% of patients. The main cutaneous lesions were classified as maculopapules (28 patients), plaques (31), LP (6), scar sarcoidosis (7) and subcutaneous sarcoidosis (14). Erythema nodosum (EN) was seen in 30 patients. Radiological stage was 0 for 8 patients, I for 48, II for 24, III for 5 and IV for 1. Systemic sarcoidosis activity persisted for > 2 years in 47 patients, and 42 received systemic corticosteroid treatment for their disease. Maculopapular and subcutaneous sarcoidosis were mainly seen in patients with EN and radiological stage I. Plaques and LP were associated with chronic disease and requirement for systemic corticosteroids. CONCLUSIONS: Cutaneous granulomatous lesions are usually present at the diagnosis of systemic sarcoidosis, and the type of cutaneous involvement may have prognostic significance.

[Lupus pernio. A report of a series of 8 patients]. / Lupus pernio. Presentación de una serie de 8 pacientes.

OBJECTIVES: Although lupus pernio (LP) is the most characteristic cutaneous lesion of chronic sarcoidosis, only a few cases have been reported in our country. The aim of this study was to review the frequency and clinical characteristics of patients with LP in a large series of patients with sarcoidosis. METHODS: A retrospective review of the frequency and characteristics of patients diagnosed as having LP from the series of sarcoidosis of our institution for a period of 35 years was performed. RESULTS: Eight (1.6%) out of 507 patients with sarcoidosis were diagnosed of LP. Mean age was 42 years. In 6 patients, LP was the presentation form of sarcoidosis. Five patients had involvement of the nasal skin and one patient severe involvement of the nasal mucosa. All the patients were treated with antimalarial drugs, 4 with oral corticosteroids, 2 with laser therapy, or with combinations with other drugs. None of the patient having nasal skin involvement showed remission of LP. CONCLUSIONS: LP is a rare clinical form of sarcoidosis in our country. No treatment is effective for nasal skin involvement. The recent introduction of infliximab may represent an advance in the treatment of LP.

Lesiones cutáneas específicas en pacientes con neurosarcoidosis / Specific Cutaneous Lesions in Patients With Neurosarcoidosis

La sarcoidosis del sistema nervioso (neurosarcoidosis [NS]) es poco frecuente, pero puede ser grave y producir secuelas importantes. Analizamos los tipos de lesión cutánea específica en pacientes con NS y su utilidad para el diagnóstico de la enfermedad. De un total de 58, 16 presentaron lesiones cutáneas específicas (27,6%). De ellos, 14 eran mujeres y dos varones (edad mediana 50 años [rango 20-84]), que presentaron 24 tipos de lesiones neurológicas (siete neuropatía craneal, cuatro parenquimatosa, tres meníngea, tres mielopatía, uno hipofisaria, dos hidrocefalia y cuatro neuropatía periférica) y 20 lesiones cutáneas específicas (seis máculo-pápulas, nueve placas, uno lupus pernio y cuatro sarcoidosis de las cicatrices [dos pacientes con máculo-pápulas y dos con placas presentaron también sarcoidosis de las cicatrices]. Las lesiones cutáneas estaban presentes al diagnóstico de la NS en 13 casos. Ante la sospecha de esta enfermedad hay que descartar la presencia de lesiones cutáneas específicas cuya biopsia puede acelerar el diagnóstico. (AU)

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis. (AU)

[Translated article] Specific Cutaneous Lesions in Patients With Neurosarcoidosis / Lesiones cutáneas específicas en pacientes con neurosarcoidosis

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis. (AU)

La sarcoidosis del sistema nervioso (neurosarcoidosis [NS]) es poco frecuente, pero puede ser grave y producir secuelas importantes. Analizamos los tipos de lesión cutánea específica en pacientes con NS y su utilidad para el diagnóstico de la enfermedad. De un total de 58, 16 presentaron lesiones cutáneas específicas (27,6%). De ellos, 14 eran mujeres y dos varones (edad mediana 50 años [rango 20-84]), que presentaron 24 tipos de lesiones neurológicas (siete neuropatía craneal, cuatro parenquimatosa, tres meníngea, tres mielopatía, uno hipofisaria, dos hidrocefalia y cuatro neuropatía periférica) y 20 lesiones cutáneas específicas (seis máculo-pápulas, nueve placas, uno lupus pernio y cuatro sarcoidosis de las cicatrices [dos pacientes con máculo-pápulas y dos con placas presentaron también sarcoidosis de las cicatrices]. Las lesiones cutáneas estaban presentes al diagnóstico de la NS en 13 casos. Ante la sospecha de esta enfermedad hay que descartar la presencia de lesiones cutáneas específicas cuya biopsia puede acelerar el diagnóstico. (AU)

A common haplotype of the C-C chemokine receptor 2 gene and HLA-DRB1*0301 are independent genetic risk factors for Löfgren's syndrome.

AIM: Sarcoidosis is a heterogeneous disorder with a strong genetic influence. Genetic factors are also thought to influence disease severity and outcome. We sought to determine whether polymorphisms within CCR2 gene predispose to Löfgren's syndrome--a clinically and genetically distinct sarcoidosis phenotype--and, importantly, whether this association is independent of the known association with the HLA-DRB1*0301 allele. METHODS: We investigated 5 CCR2 variants and HLA-DRB1*0301 by sequence-specific primer (SSP) polymerase chain reaction (PCR) in 176 Spanish (76 Löfgren's syndrome, 100 controls) and 387 Swedish subjects (126 Löfgren's syndrome, 77 non-Löfgren sarcoidosis, 184 controls). RESULTS: One of the deduced haplotypes (CCR2 haplotype 2) was associated with Löfgren's syndrome in both Spanish (OR: 2.03, uncorrected P = 0.02; permuted P = 0.041 vs. controls) and Swedish patients (OR: 3.02, uncorrected P = 0.0007; permuted P = 0.0027 vs. non-Löfgren sarcoidosis; OR: 2.46, uncorrected P = 0.0005; permuted P = 0.0031 vs. controls). HLA-DRB1*0301 allele frequency was also increased in Spanish (OR: 3.52, P = 0.0004 vs. controls) and Swedish patients with Löfgren's syndrome (OR: 10.98, P < 0.0001 vs. non-Löfgren sarcoidosis, OR: 7.71, P < 0.0001 vs. controls). Finally, multivariate analysis revealed that the CCR2 association was independent of HLA-DRB1*0301 in both Spanish (P = 0.02 vs. controls) and Swedish cohorts (P = 0.002 vs. non-Löfgren sarcoidosis, P = 0.001 vs. controls). CONCLUSIONS: This study confirms that CCR2 haplotype 2 and HLA-DRB1*0301 are independent genetic risk factors for Löfgren's syndrome.

Low levels of high density lipoprotein cholesterol in patients with active sarcoidosis.

OBJECTIVE: To determine lipoprotein abnormalities in patients diagnosed with sarcoidosis and their relation to disease activity. METHODS: We studied 90 patients with biopsy-proven sarcoidosis who had not been treated with corticosteroids (44 with active disease and 46 with inactive disease) and 147 control subjects. Sarcoidosis activity was evaluated by means of clinical, chest X-ray, gallium-67 scan, serum angiotensin converting enzyme (peptidyl-dipeptidase A) values, and pulmonary function tests. Analysis of lipoprotein metabolism included: serum cholesterol, low density lipoprotein (LDL)-cholesterol, high density lipoprotein (HDL)-cholesterol, HDL2-cholesterol, HDL3-cholesterol, apolipoprotein A-I, apolipoprotein B, and triglyceride concentrations. RESULTS: Patients with active sarcoidosis had significantly low HDL-cholesterol concentrations (1.15 +/- 0.27 mmol/l) as compared with inactive sarcoid patients (1.40 +/- 0.34 mmol/l) and with the healthy control subjects (1.49 +/- 0.34 mmol/l) (p = 0.00001). The decrease in the HDL-cholesterol concentrations seen in patients with active disease was due mainly to the cholesterol bound to HDL2 subfraction. Apolipoprotein A-I concentrations were significantly reduced in the patients with active disease (1.18 +/- 0.32 g/l) compared to the healthy controls (1.38 +/- 0.27 g/l) (p = 0.003). There were no significant differences in cholesterol, triglyceride, LDL-cholesterol or apolipoprotein B values among the three groups. Multivariate logistic regression analysis showed that HDL-cholesterol was the only variable independently associated with disease activity (Regression Coefficient b = -0.03; S.E. = 0.008; p = 0.0005). CONCLUSION: The decrease in HDL-cholesterol that is observed in patients with sarcoidosis is limited to those with active disease.

Influence of serum amyloid A on the decrease of high density lipoprotein-cholesterol in active sarcoidosis.

OBJECTIVE: We have previously observed low levels of high density lipoprotein (HDL) cholesterol in active sarcoidosis. The aim of this study was to analyze the role of serum amyloid A (SAA) on this lipid disorder. METHODS: Eighty five untreated sarcoid patients, 40 with active disease and 45 with inactive disease, were recruited. Sarcoidosis activity was evaluated by means of clinical, chest X-ray, gallium-67 scan, serum angiotensin converting enzyme (peptidyl-dipeptidase A) values, and pulmonary function tests. Analysis of lipoprotein metabolism included: serum cholesterol, low density lipoprotein (LDL)-cholesterol, HDL-cholesterol, HDL(2)-cholesterol, HDL(3)-cholesterol, apolipoprotein A-I (apo A-I), apolipoprotein B (apo B), and triglyceride concentrations. Serum amyloid A protein and lecithin-cholesterol acyltransferase (LCAT) activity were measured. RESULTS: In active sarcoidosis we found significantly reduced levels of HDL-cholesterol (1.17+/-0.36 vs. 1. 44+/-0.39 mmol/l, P=0.002), HDL(3)-cholesterol (0.78+/-0.23 vs. 1. 02+/-0.21 mmol/l, P<0.0001), and apo A-I (1.36+/-0.29 vs. 1.61+/-0. 27 g/l, P<0.0001) and significantly increased levels of triglyceride (1.51+/-0.64 vs. 1.03+/-0.46 mmol/l, P<0.0001), and apo B (1.14+/-0. 25 vs. 0.99+/-0.27 g/l, P=0.012) versus inactive sarcoidosis. Serum amyloid A concentrations were significantly increased in the patients with active disease (155.45+/-154.01 mg/ml) compared to the inactive sarcoid patients (89.70+/-65.36 mg/ml) (P=0.011). There were no significant differences in cholesterol, LDL-cholesterol, HDL(2)-cholesterol or LCAT values between groups. Multivariate logistic regression analysis showed that HDL-cholesterol (regression coefficient b=-1.96; S.E.=0.87; P=0.02) and SAA (regression coefficient b=0.01; S.E.=0.004; P=0.01) were the two variables independently associated with disease activity. Moreover, a significant negative correlation was observed between SAA levels and both HDL-cholesterol (r=-0.39; P=0.01) and apo A-I (r=-0.35; P=0.03) levels, in the active sarcoid group. Conversely, no correlation was found in the inactive sarcoid group. CONCLUSION: The low HDL-cholesterol and apo A-I concentrations seen in active sarcoid patients are associated with a significant increase of SAA levels. We suggest that the displacement of apo A-I by SAA on HDL accounts for the lower level of HDL-cholesterol seen in active sarcoidosis.

Löfgren's syndrome revisited: a study of 186 patients.

PURPOSE: To evaluate the clinical features, the results of noninvasive tests and biopsies, and the outcome of patients with Löfgren's syndrome. SUBJECTS AND METHODS: Patients diagnosed as having Löfgren's syndrome at a university hospital in Barcelona, Spain, from 1974 to 1996, were prospectively followed. Löfgren's syndrome was defined as the association of erythema nodosum or periarticular ankle inflammation with unilateral or bilateral hilar or right paratracheal lymphadenopathy. RESULTS: Löfgren's syndrome was diagnosed in 186 patients. The mean age was 37 +/- 11 years, and 157 (85%) were women. In 91 patients (49%), symptoms started during the spring (P < 0.0001). Erythema nodosum, periarticular ankle inflammation, or both were present at onset in 173 patients (93%). At the time of diagnosis, 161 patients (87%) had no respiratory symptoms; 151 (81%) had stage I abnormalities on chest radiograph, 29 (16%) stage II, and 6 (3%) stage 0. Five percent of patients had decreased forced vital capacity, and 15% had decreased carbon monoxide diffusing capacity. Extrathoracic involvement was infrequent. Serum angiotensin-converting enzyme levels were increased in 50% of patients. Gallium-67 scans showed hilar uptake in all the studied patients, but it yielded useful additional diagnostic information only in those with normal chest radiographs or with unilateral hilar lymphadenopathy. The diagnosis was proven with biopsy results in 63% of patients. None of the patients without histologic confirmation were subsequently found to have a diagnosis other than sarcoidosis. In the 133 patients who were followed for a mean of almost 5 years, 11 (8%) continued to have active disease, and 8 (6%) had several recurrences between 18 months and 20 years after a complete resolution. A normal serum angiotensin-converting enzyme level at diagnosis was associated with disease resolution without recurrence. CONCLUSION: Löfgren's syndrome is usually a self-limiting form of sarcoidosis. Histologic confirmation is not necessary in typical cases. In a small number of patients, the disease may remain active or recur long after its onset, although usually with mild organ dysfunction.

Foreign bodies in granulomatous cutaneous lesions of patients with systemic sarcoidosis.

OBJECTIVE: To assess the presence of foreign material in the granulomatous cutaneous lesions of patients with systemic sarcoidosis. DESIGN AND SETTING: Observational study reevaluating histological specimens at a university referral hospital. PATIENTS: Sixty-five patients diagnosed as having sarcoidosis who developed granulomatous cutaneous involvement. MAIN OUTCOME MEASURES: To detect the presence of polarizable foreign particles in cutaneous biopsy specimens and to evaluate the association with clinical features of the patients. RESULTS: Granulomatous cutaneous involvement was demonstrated in 65 (15.3%) of 425 patients with systemic sarcoidosis. In 14 (22%) of the 65 patients, the cutaneous biopsy specimen showed foreign particles in polarized light. The skin lesions corresponded to 3 different clinical patterns: an admixture of papules and infiltration of previously undetected minute scars (n = 6); scar sarcoidosis (n = 4); and subcutaneous nodules (n = 4). The lesions were located most frequently in the extremities, involving the knees in 10 patients. CONCLUSIONS: The presence of polarizable foreign body material in granulomatous cutaneous lesions is not infrequent in patients with systemic sarcoidosis. Inoculation of foreign matter from a previous inapparent minor trauma may induce granuloma formation in individuals with sarcoidosis.

Cutaneous involvement in sarcoidosis. Relationship to systemic disease.

Sarcoidosis is an antigen-mediated disease defined by granuloma formation in different organs. It involves mainly the mediastinal and peripheral lymph nodes, lungs, eyes, skin, liver, and spleen. Cutaneous lesions of sarcoidosis may be specific, showing histologically noncaseating granulomas, or nonspecific, most typically erythema nodosum. Frequently, both types of skin lesions are the means of presentation of the disease and may contribute to the diagnosis. A workup for systemic sarcoidosis should be undertaken in every patient with sarcoid cutaneous granulomas. Some types of cutaneous lesions have prognostic significance. Lupus pernio and plaques are associated with more severe systemic involvement and more chronic course, while erythema nodosum is the hallmark of acute and benign disease.

Nuclear imaging. 67Gallium, 201thallium, 18F-labeled fluoro-2-deoxy-D-glucose positron emission tomography.

67Gallium scan has been used for years in sarcoidosis as a marker of activity, a determiner of the extent and distribution of the disease, a diagnostic support, and an aid in therapeutic management. Because of its limited sensitivity and specificity for sarcoidosis, however, it is currently used mainly to assist in diagnosis in difficult cases, particularly in those with isolated extrathoracic sarcoidosis. The finding of the typical lambda or panda patterns supports the diagnosis and reinforces the indication to perform an appropriate biopsy or Kveim-Siltzbach test. In addition, the detection of clinically silent extrathoracic uptake may provide sites for biopsy. 67Gallium scans' routine use in the follow-up of pulmonary sarcoidosis under treatment has decreased because that is best accomplished by means of serial chest radiographs and PFT. 201Thallium scintigraphy studies the myocardial perfusion and is complementary to echocardiography and 24-hour electrocardiographic monitoring in the assessment of sarcoid cardiac involvement. It typically shows segmental areas of decreased uptake in the ventricular myocardium that disappear or decrease in size during stress or after intravenous administration of dipyridamole. That reverse distribution is not specific for cardiac sarcoidosis, however, because it may also occur in other cardiomyopathies. PET is based on the increase of glucose metabolism in inflamed tissues. It may have great potential to assess sarcoidosis activity, but it is still largely experimental and is not routinely employed.

Epidemiology of sarcoidosis in Catalonia: 1979-1989.

For the purpose of estimating the incidence rate and clinico-epidemiological characteristics of sarcoidosis in Catalonia (a country comprising the provinces of Barcelona, Tarragona, Lleida and Girona, with a total population of 6 million people), we did a retrospective study of new cases diagnosed in the country's qualified hospitals during 1979 and the three-year period from 1986 to 1988. The annual incidence rate in the province of Barcelona was 1.2/100,000 in 1979 and 1.5/100,000 in 1988. 245 cases were diagnosed in Catalonia in the three-year period covering 1986-88, the annual incidence rate being 1.36/100,000 inhabitants. We describe here the characteristics of this series of 245 cases (which is the first one representative of the whole of Catalonia), and compare them with those of other series published previously. We conclude that the incidence of sarcoidosis in our country is one of the lowest in Europe, with no significant variations observed over ten years. The most relevant clinico-epidemiological characteristics are its clear predominance in women (3:1), and the high frequency of Löfgren's syndrome (48% of cases), whose incidence is significantly higher in springtime.

Prophylactic cefamandole in orthopaedic surgery.

Three hundred patients were included in a prospective randomized double-blind trial comparing the efficacy of cefamandole with that of a placebo for prophylaxis of sepsis in operations using Ender or Küntscher nails, bone plates, or other internal fixation devices. Patients with an open fracture, total joint replacement, or direct operation on the hip were not included in the study. Sixteen patients were excluded because the trial protocol was not followed exactly, so a total of 284 patients participated, 134 of whom were given cefamandole and 150, a placebo. The two groups were similar in terms of mean age, sex ratio, duration of preoperative hospital stay, underlying risk factors, and type of surgical procedure. A superficial wound infection developed in none of the 134 patients who were given cefamandole and in seven of those in the control group (p less than 0.05). Two deep-wound infections developed in the cefamandole-treated group and four, in the control group (p greater than 0.05). Staphylococcus aureus, Staphylococcus epidermidis, and gram-negative bacilli were the most common infecting organisms. The rates of infection-related mortality and abscopal infection were similar in both groups. No adverse side effects of the drug were encountered.

Vertebral and rib sarcoidosis: long-term clinical remission with methotrexate.

We describe a patient with bilateral hilar lymphadenopathy shown on a chest radiograph and supraclavicular lymphadenopathy. Biopsy of a supraclavicular lymph node showed non-caseating granulomas. A diagnosis of sarcoidosis was made and no treatment was given. One year later she complained of cervical and lumbar pain and decreasing strength of the right hand. Magnetic resonance imaging of the spine showed multiple lesions within the vertebral bodies of six vertebrae, and thoracic computed tomography showed partial destruction of the first right rib. A biopsy of the second lumbar vertebra demonstrated non-caseating granulomas. Corticosteroid treatment was unsuccessful and long-term remission of the symptoms was achieved with a weekly low dose of methotrexate.

[Predictive factors of the persistence of sarcoidosis activity 2 years after diagnosis]. / Factores predictivos de persistencia de activad de la sarcoidosis a los 2 años del diagnóstico.

BACKGROUND: The prognosis of sarcoidosis is difficult to predict. In general, sarcoidosis is considered as chronic when it remains active for over 2 years. The aim of this study was to analyze predictive factors in the persistence of sarcoidosis activity 2 years after diagnosis. METHODS: One hundred fifty-five patients diagnosed of having sarcoidosis over a period of 14 years were included in a protocol of prospective study and were followed for at least 2 years. RESULTS: Four variables independently related to the persistence of activity of the disease at 2 years of diagnosis were identified by means of multiple logistic regression analysis: absence of erythema nodosum (relative risk = 23.3), confidence interval 95% = 12.7-44.6), age equal to or greater than 40 years of age (RR = 7.7, IC = 4.3-13.6), presence of splenomegaly (RR = 21.1, IC = 7, 5-59.7) and presence of parenchymatous infiltrates in thorax x-ray (RR = 3.5, IC 2-5.9). CONCLUSIONS: Although the persistence in the activity of sarcoidosis does not always have the same clinical repercussion and does not necessarily imply treatment, these results may permit a greater approximation to the prognosis of the disease and the indication of corticotherapy.

[The Kveim-Siltzbach test in sarcoidosis]. / La prueba de Kveim-Siltzbach en la sarcoidosis.

BACKGROUND: The Kveim-Siltzbach test has been widely used in the diagnosis of sarcoidosis. This reactive is currently available in the authors' hospitals. Previous experience with this test in the same centers was reviewed to know its diagnostic usefulness. The present is the first series described in Spain. METHODS: From 1977 to 1988 the Kveim-Siltzbach test was performed in 79 patients diagnosed or suspected of having sarcoidosis. The study was carried out as cooperation of the validation process of sarcoid spleen suspensions (lots K12, K12 1/2, K32, K41, K42, K42 1/2 and K50 of the Colindale antigen) prepared in the Standards Laboratory for Serological Reagents in the Royal Brompton Hospital in London. RESULTS: The results of test positivity were determined following the criteria established by Siltzbach. The global sensitivity of the Kveim-Siltzbach test was 78% (CI 95% = 67.8-86.9). Positivity ranged from 84% in subacute sarcoidosis to 61% in chronic sarcoidosis (p = 0.08). Likewise, it was higher in patients with radiologic stage I (84%) and II (81%) with respect to stage III (62%) or the cases with exclusive extrathoracic sarcoidosis (stage 0) (57%) (p = 0.11). No association was found between the Kveim-Siltzbach test positivity and the elevation in serum values of the angiotensin-converting enzyme (p = 0.575). CONCLUSIONS: In the present study the Kveim-Siltzbach test showed high sensitivity in the diagnosis of sarcoidosis. This fact, together with the high specificity observed in other studies, makes the test a very useful tool for the non-invasive diagnosis of sarcoidosis.