Alelos HLA na contratura de Dupuytren

A Contratura de Dupuytren (DC) é uma doença fibroproliferativa caracterizada pela formação de nódulos e cordões fibrosos que se retraem longitudinalmente em direção aos dedos causando à contratura digital que determina o aspecto de mão em garra, sendo por isso citada como diagnóstico diferencial da hanseníase. Sua incidência é maior na raça branca de origem européia, principalmente em adultos do sexo masculino com idade superior a 50 anos. Embora existam evidências do envolvimento genético no aparecimento da DC, até o momento nenhum gene foi associado como fator de risco para a doença.

Neurophysiological patterns of ulnar nerve neuropathy in leprosy reactions.

BACKGROUND: Leprosy neuropathy, despite being primarily demyelinating, frequently leads to axonal loss. Neurophysiological examination of the nerves during Type 1 (T1R) and Type 2 reactions (T2R) may give some insight into the pathophysiological mechanisms. METHODS: Neurophysiological examinations were performed in 28 ulnar nerves during a clinical trial of steroid treatment effectiveness, 19 patients with T1R and nine with T2R. The nerves were monitored during a period of 6 months; there were eight assessments per nerve, for a total of 224 assessments. Nine neurophysiological parameters were assessed at three sites of the ulnar nerve. The compound motor action potential amplitudes elicited at wrist, elbow and above, as well as the conduction velocity and temporal dispersion across the elbow, were chosen to focus on the changes occurring in the parameters at the elbow tunnel. RESULTS AND CONCLUSION: Neurophysiological changes indicating axonal and demyelinating processes during both T1R and T2R were detected across the elbow. Changes in demyelination, i.e. a Conduction Block, as a primary event present during T2R, occurring as an acute phenomenon, were observed regularly; in T1R Temporal Dispersion, a subacute phenomenon, was seen. During treatment remyelination occurred after both types of reactions.

Utility of measuring serum levels of anti-PGL-I antibody, neopterin and C-reactive protein in monitoring leprosy patients during multi-drug treatment and reactions.

OBJECTIVE: To verify the validity of measuring the levels of Mycobacterium leprae-specific anti-phenolic glycolipid (PGL)-I antibody, neopterin, a product of activated macrophages, and C-reactive protein (CRP), an acute phase protein, in serial serum samples from patients for monitoring the leprosy spectrum and reactions during the course of multi-drug treatment (MDT). METHODS: Twenty-five untreated leprosy patients, 15 multi-bacillary (MB) and 10 paucibacillary (PB), participated. Eight patients developed reversal reaction and five developed erythema nodosum leprosum (ENL) during follow-up. The bacterial index (BI) in slit-skin smears was determined at diagnosis and blood samples collected by venipuncture at diagnosis and after 2, 4, 6 and 12 months of MDT. PGL-I antibody and neopterin were measured by enzyme-linked immunosorbent assay, whereas the CRP levels were measured by the latex agglutination method. RESULTS: The levels of PGL-I antibodies and neopterin were higher in the sera of MB than PB patients, which correlated with the patients' BI. The serum levels of CRP did not differ significantly between the MB and PB patients. The serum levels of PGL-I and neopterin were no higher in reactional patients than non-reactional patients prone to such reactions. However, ENL patients had higher serum CRP levels than non-reactional MB patients. The serum PGL-I antibody levels declined significantly during MDT, in contrast to neopterin and CRP levels. CONCLUSION: Measuring the serum levels of PGL-I antibodies and neopterin appeared to be useful in distinguishing MB from PB patients, whereas monitoring the levels of PGL-I antibodies appeared to be useful in monitoring MB patients on MDT. Measuring serum CRP, although not useful in monitoring the patients, has limited significance in detecting ENL reactional patients.

Renal lesions in leprosy: a retrospective study of 199 autopsies.

In the present work, 199 patients with leprosy who underwent autopsy between 1970 and 1986 were retrospectively studied to determine the prevalence, types, clinical characteristics, and etiologic factors of renal lesions (RLs) in leprosy. Patients were divided into two groups: 144 patients with RLs (RL+) and 55 patients without RLs (RL-). RLs observed in 72% of the autopsied patients were amyloidosis (AMY) in 61 patients (31%), glomerulonephritis (GN) in 29 patients (14%), nephrosclerosis (NPS) in 22 patients (11%), tubulointerstitial nephritis (TIN) in 18 patients (9%), granuloma in 2 patients (1%), and other lesions in 12 patients (6%). AMY occurred most frequently in patients with lepromatous leprosy (36%; nonlepromatous leprosy, 5%; P < 0.01), recurrent erythema nodosum leprosum (33%; P < 0.02), and trophic ulcers (27%; 0.05 < P < 0.10). Ninety-seven percent of AMY was found in patients with lepromatous leprosy, 88% showed recurrent trophic ulcers, and 76% presented with erythema nodosum leprosum. NPS was found in older patients with arterial hypertension, neoplastic diseases, infectious diseases, and vasculitis associated with GN. Most patients with AMY presented with proteinuria (95%) and renal failure (88%). The most frequent causes of death were renal failure in patients with AMY (57%), infectious diseases in patients with GN (41%) and TIN (45%), and cardiovascular diseases in patients with NPS (41%). No difference in survival rates was observed among RL- patients and those with AMY, GN, NPS, or TIN.

No evidence of linkage between Mitsuda reaction and the NRAMP1 locus.

Thirty sib-pairs were ascertained through unrelated lepromatous probands. They consisted of 22 healthy individuals and 8 leprosy patients. The Mitsuda reactions of all sibs were evaluated both macroscopically and histologically, and high molecular weight genomic DNA was extracted from the white blood cells of all sib-pairs. Three DNA polymorphisms identified by polymerase chain reaction (274C/T, D543N, 1729 + 55del4) were used as chromosome markers at the NRAMP1 locus. Sib-pair comparisons did not disclose any sign of close linkage between the Mitsuda reaction and the genetic markers.

Anti-neutrophil cytoplasmic antibodies (ANCA) in the clinical forms of leprosy.

Anti-neutrophil cytoplasmic antibodies (ANCA) are autoantibodies against enzymes present in primary granules of neutrophils and lysosomes of monocytes detected in systemic vasculitis and in other diseases, including infections. ANCA are markers of active Wegener granulomatosis, which presents some anatomo-pathologic and immune response features similar to those of leprosy. Thus, we raised the hypothesis that ANCA may be present in leprosy as markers specifically linked to the presence of vasculitis. The aim of this study was to determine the presence of ANCA in leprosy and its correlation with the clinical forms of the disease. Sera from 60 normal individuals and from 59 patients with different clinical forms of leprosy were studied. The patients were also allocated into reactional and nonreactional groups. By indirect immunofluorescence, ANCA were positive, an atypical pattern (A-ANCA), in 28.8% of the patient sera. A-ANCA predominated, although not significantly (p > 0.05), in the reactional groups 37.9% vs 20.0%), and in those at the lepromatous pole (41.6% vs 20.0%). There was no correlation between ANCA positivity and either disease duration, disease activity, or therapeutic regimen (p > 0.05). An interesting finding was the correlation between ANCA and gender: 94.1% of ANCA-positive patients were males (p < 0.01), a feature that so far has not been reported in ANCA-related diseases and for which there is no explanation at the moment. By ELISA, the sera of the lepromatous leprosy patients did not show activity against either PR3, MPO, HLE, the most common ANCA antigens. Because A-ANCA are nonspecific, this finding requires further investigation for the determination of the responsible antigen(s). In conclusion, A-ANCA are present in 28.8% of leprosy patients but are not related to vasculitis in the erythema nodosum leprosum reaction and are not a marker of a specific clinical form.

[Assessment of the Mitsuda reaction in lepromatous patients inactive before and after immunotherapy]. / Avaliação da reação de Mitsuda em pacientes virchovianos inativos antes eapós imunoterapia.

In this study the immunopotentiator levamisole as well as a mixture of BCG/Mycobacterium leprae were investigated in inactive lepromatous leprosy patients by using the Mitsuda reaction as a parameter. Twenty lepromatous patients ten years ago classified as histologically negative for Mitsuda's test were divided into three groups: five patients that were only retested with Mitsuda antigen; eight patients that received oral levamisol and seven patients that received a mixture of alive BCG plus autoclaved M. leprae. The results indicated that: 1) the levamisole did not alter the reactivity to lepromin in any of the patients studied; 2) neither the changes in the reactivity to lepromin by using the mixture (3 cases) nor those that occurred spontaneously (3 cases) were clear. They properly reflected the natural variation of patients with some degree of resistance to Mycobacterium leprae.

Oral lesions in borderline and reactional tuberculoid leprosy.

Thirty patients, fifteen with borderline and fifteen with reactional tuberculoid leprosy, were submitted to clinical and histopathologic studies of the buccal mucosa for detection of specific lesions. Five reactional tuberculoid and eight borderline patients presented specific conditions characterized by chronic granulomatous lesions with bacilli, chronic granulomatous lesions without bacilli, and nonspecific chronic inflammatory lesions with bacilli. The infiltrate had small extension, low bacterial levels and the mucosa, with the exception of one case, did not show ulceration. These results suggest that in the reactional tuberculoid and borderline patients the buccal mucosa is not an important source of bacilli elimination.

[The Rubino reaction. Criterion for inactivation in lepromatous patients]. / Reação de Rubino. Critério de branqueamento para pacientes virchowianos.

Approximately 55% of active lepromatous patients respond positively to the Rubino reaction. With arrested cases this rate of positivity lowers considerably to about 15%. In an effort to associate this reaction with the presence of bacilli, a study of 796 cases was undertaken. The patients were divided into: a control group, active tuberculoid cases, arrested tuberculoid cases, active borderline cases, arrested borderline cases, active lepromatous cases, and arrested lepromatous cases. The patients were submitted to the following tests: Rubino reaction, presence of cryoglobulins, and VDRL and PCR positivity. By the results obtained we may conclude that: a) A positive Rubino reaction may be present in all the forms of leprosy studied, this reaction having an inverse relationship with the organism's resistance to "M. leprae". b) The Rubino reaction has specificity to leprosy. c) This reaction does not depend on the number of bacilli present in the host. d) A positive Rubino reaction is not related to the presence of cryoglobulins in the serum, nor to VDRL or PCR positivity, nor to the length of time the patient's disease has been arrested. The authors present these findings and suggest that this reaction be used as one of the criteria for determining cure.

[Developmental aspects of the Rubino reaction]. / Aspectos evolutivos de reação de Rubino.

The Rubino reaction was studied in 178 leprosy patients to correlate its positivity, clinical form, E.N.L., and time and activity of the disease. The patients studied were classified according to the criteria established by the 1953 Madrid Congress. The results show that the tuberculoid patients presented negative Rubino reactions regardless of any of the parameters studied. The borderline patients showed 9% positive Rubino reaction not related to any of the observed parameters studied. The lepromatous patients showed a significant difference of positivity to the Rubino reaction when grouped as to the disease's active or non-active presence. Active lepromatous patients showed 55% of positivity to the Rubino reaction whereas patients considered clinically inactive showed only 6% of positivity. The fact that the Rubino reaction is negative in the majority of patients who are clinically cured would indicate that this test could be included as part of the criteria used in determining which clinically cured lepromatous patients can discontinue medication without risking a relapse.

[Cellular immunity in leprosy patients]. / Estudo da imunidade celular em pacientes branqueados.

Forty eight clinically arrested lepromatous patients were submitted to several tests to evaluate cellular immunological competence (PPD, Trichophytin, DNCB, determination of the percentage of T lymphocytes in the peripheral blood, and early and late reactions to the lepromin). The results of the Trychophytin test and of the lymphocyte percentage test did not differ from those of the control group, whereas the results to the PPD test were more often positive for the arrested Virchovian cases. All the patients presented positivity to the DNCB test, and none of them showed positivity to the Fernandez reaction. As for the late reaction to the lepromin, all the patients were negative, although in histopathological testing four degrees of reactivity was observed ranging from focal, non specific inflammatory reaction to granulomatous histiocytic with an epithelioid outline. By means of these results the authors were able to confirm that: 1. There is no cross reactivity between the PPD and lepromin tests. 2. Sulfone therapy does not interfere in immunocellular results for the tests employed. 3. Lepromatous patients respond like the general population to the unspecific sensitivity tests utilized. 4. The finding of histiocytic reaction with epithelioid outline in some biopsies of Mitsuda reaction in lepromatous patients, suggests the presence of a sub-population within this group, probably Borderline patients that suffered during the infection's evolution downgrading of theirs clinic and histological characteristics.

Reacao de tipo pseudo-exacerbacao e carcinoma bronquiolar dos pulmoes (conferencia clinica). / Pseudoexacerbation reaction and bronchiolar carcinoma (clinical conference)

Um paciente, portador de infecao hansenica ha 20 anos, e considerado como virchowiano inativado, apresentou nos ultimos 4 anos de vida, dois surtos de reacao de pseudoexacerbacao (reacao reversa), o ultimo deles coincidindo com internacao onde foi diagnosticado um carcinoma bronquiolar dos pulmoes, cujo agravamento ocasionou o obito. Discutem-se as dificuldades do diagnostico clinico diferencial deste tipo de carcinoma pulmonar com outras patologias com caracteristicas clinicas e evolutivas semelhantes, bem como a dificuldade de se chegar a este diagnostico apenas pela analise de uma biopsia pulmonar. Em relacao as reacoes de pseudo-exacerbacao, duas interpretacoes sao postas em confronto, e e salientada a hipotese destas reacoes se desenvolverem como resposta a surtos de proliferacao bacilar que podem ocorrer em pacientes dimorfos em qualquer fase da evolucao da infeccao, devido a tratamento inadequado ou resistencia medicamentosa.Tambem analisa-se a eventual relacao do ultimo surto reacional com alteracoes da imunidade celular secundarias ao desenvolvimento da neoplasia pulmonar