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PubMedDate not supplied

Cell and humoral immunity in endemic pemphigus foliaceus.

A study was conducted on 16 patients with pemphigus foliaceus, ten of them with the localized form (group G1) and six with the disseminated form (group G2). These patients were submitted to full blood counts, quantitation of mononuclear cell subpopulations by monoclonal antibodies, study of blastic lymphocyte transformation, and quantitation of circulating antibodies by the indirect immunofluorescence test, in order to correlate their clinical signs and symptoms and laboratory data with their immunological profile, and to determine the relationship between circulating autoantibody titers and lesion intensity and course of lesions under treatment. Leucocytosis was observed especially in group G2. All patients showed decreased relative CD3+ and CD4+ values and a tendency to decreased relative values of the CD8+ subpopulation. Blastic lymphocyte transformation indices in the presence of phytohemagglutinin were higher in patients (group G1 + G2) than in controls. The indirect immunofluorescence test was positive in 100% of G2 patients and in 80% of G1 patients. The median value for the titers was higher in group G2 than in group G1. Analysis of the results as a whole permits us to conclude that cell immunity was preserved and that there was a relationship between antibody titers detected by the direct immunofluorescence test and extent of skin lesions.

Adolescent↗
PubMedDate not supplied

[Evaluation of the polymorphisms in methylenetetrahydrofolate reductase gene and the levels of folate and B12 in HIV-infected patients under antiretroviral therapy].

In this study we sought to investigate the B12 and folate levels regarding the influence of methylenetetrahydrofolate reductase genotypes, immunological profile and antiretroviral therapy in the Brazilian HIV-infected population. The study group comprised 89 HIV-infected individuals and 29 blood donors. There was a decrease in the B12 levels in the HIV-infected group with higher TCD4+ lymphocyte counts. No differences in the genotype distribution for methylenetetrahydrofolate reductase polymorphisms between the HIV-infected individuals and the controls were found. HIV-infected individuals carrying the C677C genotype presented lower B12 levels (313.91 +/- 154.05) than those with the same genotype in the control group (408.27 +/- 207.69). Also, the antiretroviral therapy was not a source of variation of the folate and B12 serum levels. Further studies are needed to reanalyze the prevalence of low levels of folate and B12 and hyperhomocysteinemia among HIV-infected patients with regard to the use of HAART and the increased life expectancy of such patients.

Adult↗
PubMed1996

Immunological, genetic, and microbiological study of family members manifesting early-onset periodontitis.

From the standpoint of host-parasite interactions, family studies help us understand the host defensive factors and the molecular mechanisms involved in the periodontal immune response. In this study, we report the immunological profile of host-defensive functions, human leukocyte antigen (HLA) phenotypes, and the microflora of a mother (rapidly progressive periodontitis), an older son (periodontally healthy), a younger son (localized juvenile periodontitis), and a daughter (localized juvenile periodontitis). We examined the peripheral neutrophil functions, phenotypic and functional analysis of peripheral lymphocytes, serum immunoglobulin G (IgG) antibody titers against periodontopathic bacteria, serological type of HLA class II antigens, and bacterial flora in all periodontal pockets. The results showed that Actinobacillus actinomycetemcomitans was dominant in the pockets of all subjects. The mother and two sons showed a depressed neutrophil chemotaxis to N-formyl-methionyl-leucyl-phenylalanine. All subjects except the older son exhibited low T4/T8 ratios. The mother and daughter had raised levels of IgG titers to Porphyromonas gingivalis. All subjects had HLA phenotypes of DRw52 and DQ1 in common. We found that the family members had similar disorders in certain defensive functions. This family has been a model for our understanding of the host defensive factors in the development of early-onset periodontitis.

Adolescent↗
PubMed2001

Treatment of aggressive periodontitis by osseointegrated dental implants. A case report.

BACKGROUND: Generalized aggressive periodontitis is described as a clinical entity affecting both deciduous and permanent dentition with extensive alveolar bone loss, mobility, and exfoliation of all or many teeth. Controversy exists on dental implant use to restore missing dentition in younger patients. METHODS: This case report presents a patient diagnosed with aggressive periodontitis who has lost all but 4 of her teeth. Her personal and functional desires led us to include implant therapy in her treatment plan. The hematological data are presented with an analysis of the immunological profile. RESULTS: Dental implants were placed, and following 3 months of osseointegration, an implant-supported prosthesis was completed. The patient was followed up for 36 months. CONCLUSIONS: This case report presents an alternative treatment for rehabilitating dentition in a young patient treated for aggressive periodontitis. Similar case studies may help eliminate some of the controversy that exists regarding the use of dental implants in aggressive periodontitis patients.

Adolescent↗
PubMed2003

Secondary infections in patients with atopic dermatitis.

Clinicians have long since been aware that bacteria and other microorganisms play a role in the etiology of atopic dermatitis. Indeed, the immunological profile of atopy favors colonization by Staphylococcus aureus, and the bacteria are present in most patients with atopic dermatitis, even in the absence of skin lesions. Clinical signs of impetiginization, such as weeping and crusting, periauricular fissuration, or small superficial pustules are a sensitive indicator that the numbers of S. aureus may have increased and a clinical indication of secondary infected dermatitis. However, recent research that has focussed on the role of S. aureus in atopic dermatitis, offers a reversed perspective, by presenting evidence that the underlying pathology of atopic dermatitis, i.e. an alteration of the skin barrier and inflammation of the upper dermis, depends itself on the presence of an infectious process. In other words, secondary infection with S. aureus emerges as a cause of atopic dermatitis. Secondary infections due to fungi have, comparatively, received less attention, but there is evidence for a role for Malassezia spp. as a factor in dermatitis with a head and neck distribution pattern. Viral infections, such as herpes simplex virus, and mixed infections of intertriginous spaces, may complicate an underlying atopic dermatitis, but are not perceived as etiologic factors. Recent research has greatly contributed to our understanding of the pathophysiological potential of S.aureus superantigens in atopic dermatitis, suggesting that antibiotic therapy might be an important element in the therapeutic management of atopic dermatitis. At present, however, the clinical evidence is scarce with regards to demonstrating a clear advantage of combined anti-inflammatory and antibiotic treatment, compared with anti-inflammatory treatment alone. If there is a consensus that the presence of clinically infected lesions in atopic dermatitis warrants a course of specific antibiotic topical therapy, the clinical benefit of antibiotic agents in apparently uninfected atopic dermatitis, as present in the majority of patients, remains an open question.Moreover, the impact of adjuvant skin care on the cutaneous microflora needs to be quantified in order to properly assess the role of specific antibiotic therapy in clinically uninfected atopic dermatitis. In the meantime, secondary infections in atopic dermatitis remain a secondary problem in clinical atopic dermatitis management, and specific anti-infective therapy remains a method of fine-tuning for optimizing individual atopic dermatitis treatment.

Adrenal Cortex Hormones↗
PubMed2003

Effect of long-term exposure to insulin lispro on the induction of antibody response in patients with type 1 or type 2 diabetes.

OBJECTIVE: To determine the long-term effects of insulin lispro on inducing lispro-specific, insulin-specific, and cross-reactive (reactive with both insulin lispro and human insulin) antibodies. RESEARCH DESIGN AND METHODS: A multinational, multicenter combination of controlled and noncontrolled, open-label studies of 4.5 years' duration was designed to evaluate the long-term immunologic profile of subcutaneously administered insulin lispro. A total of 1,221 patients (men and women; 12-81 years of age) with type 1 or type 2 diabetes were enrolled. Circulating anti-insulin antibodies were measured using radioimmunoassays. RESULTS: Insulin-specific and lispro-specific antibody responses were within the background noise levels of the assays. Significant elevations of antibody were confined to a cross-reactive antibody response. Antibody levels resulting from prior exposure to long- and short-acting insulins changed little after transfer to insulin lispro and remained within or near the baseline levels. De novo exposure to insulin lispro resulted in increases in cross-reactive but not insulin- or lispro-specific antibody levels. Cross-reactive insulin antibodies developed more readily in patients with type 1 diabetes than in those with type 2 diabetes. Long-term antibody responses tended to decrease over time and returned to baseline or near-baseline levels by the end of the long-term studies. No evidence of an anamnestic antibody response could be found in individuals treated intermittently with insulin lispro. CONCLUSIONS: The immunogenic profile of patients treated with insulin lispro was comparable to that of patients treated with recombinant human insulin. Inductions of significant levels of specific or cross-reactive antibodies were not observed in patients who had received insulin previously. No significant antibody-dependent increases in insulin dosage requirements were noted in these patients. The incidence of insulin allergy was not different from that in patients treated with recombinant regular human insulin.

Adolescent↗
PubMed1997

The relationship between fibromyalgia and the multiple chemical sensitivity syndrome.

Fibromyalgia (FM) patients often report a high frequency of non-musculoskeletal symptoms, including those suggestive of multiple chemical sensitivity (MCS) syndrome. The objective of this pilot study was to determine the prevalence of MCS in FM patients from a university-based rheumatology practice. Self-report questionnaires were administered to determine the presence of MCS, utilizing the criteria from a recent study of the immunologic profile of patients with this disorder. Patients also responded "yes" or "no" to the presence of 48 FM-related symptoms. Thirty-three of 60 patients with FM met the criteria for MCS. Eleven of these patients also fulfilled more restrictive criteria, requiring a "higher degree" of chemical sensitivity. The symptoms and substances most frequently cited were similar to those reported in other studies of MCS. FM patients with and without MCS did not differ in other symptomatology. MCS may represent an additional symptom complex within the spectrum of FM.

Adult↗
PubMed1997

Decrease in the concentrations of transforming growth factor-beta 1 in the sera of patients with Kawasaki disease.

Kawasaki disease (KD) is one of the most important forms of vasculitis, and is characterized by the initiation of a proinflammatory cytokine cascade. To further characterize the immunological profile of KD, we measured the serum levels of transforming growth factor-beta 1 (TGF-beta 1) as a regulatory cytokine. We determined the concentration of TGF-beta 1 in the sera of the patients with KD, anaphylactoid purpura (AP), and scarlet fever, using a sandwich enzyme linked immunosorbent assay. The serum levels of TGF-beta 1 were decreased in patients with KD, but not in patients with AP or scarlet fever during the acute stage. We found an inverse correlation between TGF-beta 1 and soluble tumor necrosis factor (TNF) receptor levels in KD patients during the acute and subacute stage. Decreased levels of TGF-beta 1, in particular to suppress TNF alpha (TNF-alpha) production, is an important part of the regulatory system of increased TNF-alpha production which cause vasculitis.

Adolescent↗
PubMed1992

Immunomodulatory effects of gamma-HCH (Lindane) in mice.

Mice were fed for 24 weeks with three different subtoxic dosages of gamma-HCH (0.012, 0.12 and 1.2 mg/kg) mixed in powdered feed. The immunological profile was assessed at an interval of one month during the entire exposure period. Both the cell mediated and humoral components of immunity showed a biphasic response characterized initially by stimulation followed by suppression in a dose dependent manner. However, gamma-HCH did not affect the functional properties of peritoneal macrophages. Histological changes in lymphoid organs were in accordance with the biphasic immunomodulatory effects of gamma-HCH.

Animals↗
PubMed1994

Morphological and immunophenotypical characterization of Japanese B-cell lymphocytic leukemia.

We have analyzed the clinical and laboratory features of 42 patients with B-cell leukemia. Based on the FAB criteria, the cases were classified in 3 groups: I) typical CLL 15, II) atypical CLL 9 which included 6 cases with large cells, and III) B-cell lymphoma in leukemic phase 18. Cases diagnosed as typical CLL (group I) had similar features to those seen in CLL patients from Western countries. The morphology and markers in cases from group III corresponded to B-cell lymphoma in leukemic phase. On the other hand, group II included 3 cases classified as atypical CLL according to FAB criteria. 1 CLL/PL and 2 mixed CLL and 6 cases with rather distinct features, namely: 1) lymphocytosis (42 +/- 41 x 10(9)/l in average) with large mature-looking lymphocytes with abundant cytoplasm: 2) an immunological profile consistent with CLL but, in addition with the consistent expression of CD38; 3) absence of a monoclonal band in the serum and 4) a clinical course and prognosis similar to CLL. Our findings suggest the existence of a B-cell disorder in Japan very close to CLL but distinct from typical and atypical CLL as seen in Western countries. Further studies would clarify whether such an entity is exclusively confined to Japan having a distinct natural history.

Adult↗
PubMed2002

[The high frequency of spontaneous micronuclei observed in lymphocytes of systemic sclerosis patients: preliminary results].

OBJECTIVE: Aim of the study is to assess the presence of spontaneous chromosome damage in patients affected by limited (lSSc) or diffuse (dSSc) Systemic Slerosis, using the micronucleus (MN) assay. METHODS: We evaluated MN frequency in cultured peripheral lymphocytes of 18 SSc and in a group of 20 healthy controls. Patients were also classified as ACA+, Scl70+, FAN+ according to the presence of the specific anti-nuclear antibodies. We also explored the hypothesis that the extent of cytogenetic alteration might be related to the severity of the pathological condition and/or to the immunological profile. RESULTS: Compared to controls, the patient group as a whole showed significantly higher MN frequencies (10.8+/-4.5 vs. 27.8+/-13.7, p<0.001). No correlation was found between spontaneous chromosome damage and severity of the disease, being MN frequency 33.1+/-17.0 and 19.8+/-2.7 in lSSc and dSSc, respectively. Interestingly, ACA+ subjects displayed the highest MN frequency (36.9+/-15.0), as compared to patients with different antibody pattern (Scl70+, FAN+; 19.7+/-8.2). CONCLUSIONS: Our results confirm the presence of chromosomal damage in circulating lymphocytes of SSc patients and would suggest a key role of antibodies to the centromere in determining the observed cytogenetic anomalies.

Aged↗
PubMed2003

CD4T lymphocyte subsets and disease manifestation in children with and without HIV born to HIV-1 infected mothers.

OBJECTIVE: To understand the natural history of HIV-1 infection in children in terms of evolution of childhood clinical manifestations versus the immune status, we prospectively studied children with and without maternally transmitted HIV-1 infection born to mothers infected with HIV-1 for two years between March 1998 and March 2000. DESIGN: A prospective cohort study. SETTING: An institutional children's home. SUBJECTS: Fifty nine children (26 males and 33 females) with and without maternally transmitted HIV-1 infection born to mothers infected with HIV-1 and adopted in institutional children home. METHODS: HIV-1 status of children under nine months was confirmed by polymerase chain reaction(PCR). ELISA for HIV-1 antibody in serum/plasma was used to confirm HIV-infection status for children aged < or = 18 months. Children were visited every three months between March and June 2000. At every visit blood was collected for total white cell count, haemoglobin and CD4+ and CD8+ T cell counts. The institutional doctor routinely examined children and treated all ailments. Clinical data were recorded. MEASURES: HIV-DNA, anti-HIV antibodies, total white blood count, total T cell counts, CD4 and CD8 T cell subset counts, frequency of childhood manifestations of infection. RESULTS: The children were aged between 4.5 and 13 years. The baseline haematological and immunological profiles (mean, mode) were: HIV-1 sero-converters (WBC 7151,7150; HB 11.6, 12.0; CD4+ 686, 795; CD8+ 2168, 1507) and HIV-1 de-seroconverters (mean, mode) were: (WBC 8386, 7150; HB 11.7, 12.8; CD4+ 735, 795; CD8+ 2168, 1507). The commonest causes of illnesses among the HIV-1 children were URTI (85.3%), TB(56.1 %), pneumonia (56.2%), tonsillitis (34.1%), parotiditis (28%) and acute otitis media (25%). The distribution of clinical manifestations was similar between the two categories of children, except URTI, whose prevalence was significantly increased among HIV-1 infected children (p-value=0.006). Among the HIV-1 infected children, only TB, parotiditis, and acute otitis media (AOM) were significantly associated with decreased CD4+ T cell count (p<0.05) resulting from HIV infection. CONCLUSIONS: HIV infection in children predisposes them to common childhood infections that can be used as markers of immune decline. TB, AOM, URTI may be early indicators of suspicion that would enable selective screening for HIV infection in children.

Biomarkers↗
PubMed1984

Immunologic abnormalities in patients receiving multiple blood transfusions.

Concern for the potential risk of the acquired immunodeficiency syndrome among blood cell recipients led us to measure immunologic functions in patients who had received multiple transfusions. Abnormalities of two immunologic tests, natural killer cell function and helper/suppressor (T4/T8) lymphocyte subpopulation ratio, have characterized patients with the acquired immunodeficiency syndrome and are prevalent in populations at risk for this syndrome. Natural killer cell function was severely depressed in multiply transfused patients. However, T4/T8 ratios were normal in this population. The role of chronic antigenic stimulation was studied by measurement of HLA-DR expression on T cells. The expression of HLA-DR antigen is markedly elevated in multiply transfused patients. These results show that chronic exposure to foreign antigens may be associated with abnormalities of immunologic function, but that chronically transfused patients do not have the same immunologic profile as reported in some homosexuals and hemophiliacs.

Acquired Immunodeficiency Syndrome↗
PubMed1998

[Past and present studies on ABO blood group system].

The author engaged himself in the studies of ABO blood group system for the last three decades, and reviewed the progresses in this period, which were classified into following 5 items. 1. H-, A- and B-active oligosaccharides were isolated from the globoside fractions from human erythrocytes by ozonolysis. One of the H-active oligosaccharide with short carbohydrate chain is a pentasaccharide: Fuc(alpha 1-->2)Gal(beta 1-->4)GlcNAc(beta 1-->3)Gal(beta 1-->4)Glc, and the other with long carbohydrate chain is a heptasaccharide: Fuc(alpha 1-->2)Gal(beta 1-->4)GlcNAc(beta 1-->3)Gal(beta 1-->4)GlcNAc(beta 1-->3)Gal(beta 1-->4)Glc. Hexa- or octasaccharides with blood group A- or B-activity have an additional alpha-N-acetylgalactosaminyl residue or alpha-galactosyl residue, which joints with alpha 1-->3 linkage to subterminal beta-galactose of the both of H-active oligosaccharides, respectively. 2. A blood group A-gene specified alpha-N-acetyl-galactosaminyltransferase (A-enzyme) catalyzes the transfer of N-acetylgalactosamine from the UDP-sugar to the subterminal beta-galactosyl residue of blood group H-active carbohydrate chain, and a blood group B-gene specified alpha-galactosyltransferase (B-enzyme) catalyzes the transfer of galactose from the UDP-sugar to the subterminal beta-galactosyl residue of blood group H-active carbohydrate chain, respectively. Either the A- or B-enzyme can not transfer the substrate sugar to the carbohydrate chain lacking alpha-fucosyl residue of H-determinant, and it is the reason why the synthesis of blood group A- or B-antigenic structure in inhibited in the tissues of Bombay phenotype and in the secretory glands of the nonsecretor. 3. Specific antibody either to the A- or B-enzyme can be introduced in the serum of the rabbit which was immunized with the A- or B-enzyme preparation, respectively. And immunological cross reaction is also present between the A- and B-enzyme, but the immunologically cross reactive material can not be found in the blood group O individual. The absence of immunologically cross reactive material in the blood group O individual is supported by a fact that the cross reactive antibody similar to the antibody in rabbit serum was present in the serum of the chronic myeloid leukemia patient, who was belonged to blood group B and treated with blood group incompatible bone marrow transplantation from blood group O donor, because it is acceptable to speculate that the grafted lymphocytes react to the B-enzyme in the recipient and produce the anti-enzyme antibody. 4. The immunological profiles described above are compatible with the cDNA structures of human blood group ABO alleles presented by Yamamoto F. et al. Their gene model is that the cDNAs of blood group ABO alleles are highly homologous, but the cDNA of common O allele is non-functional due to a single nucleotide deletion close to the 5'end of the coding sequence, which causes a frame shift of the codon, and results in truncated peptide. 5. Transcription of the human blood group ABO gene can be enhanced by a CBF/NF-Y which binds the minisatellite on the 5'-upstream sequence of the gene.

ABO Blood-Group System↗
PubMed2000

Lymphocyte subpopulations after normal pregnancy and spontaneous abortion in primigravidas.

OBJECTIVE: To evaluate lymphocyte subpopulations after a first pregnancy in women who had normal pregnancies and in those whose pregnancies terminated in spontaneous abortion. STUDY DESIGN: Sixty healthy, nonpregnant women in three groups were studied: 20 with a prior abortion, 20 with a prior normal pregnancy and 20 nulligravid. Peripheral blood lymphocytes were studied using monoclonal antibodies and flow cytometry. Women were followed for one year, and if they became pregnant again, pregnancy complications were recorded. RESULTS: The percentage of B lymphocytes was significantly decreased in the postpartum group (6% +/- 2.22) in comparison to nulligravid women (8.7% +/- 3.37) (P = .005). The percentage of CD4 lymphocytes was significantly higher in the postabortion group (44.7% +/- 7.81) in relation to the control group (39.85% +/- 6.01) (P = .03). A significantly higher CD4/CD8 ratio was found in the postabortion group in relation to the control group (1.65 vs. 1.24) (P = .01). Women with pregnancy complications in their next pregnancy had a lower absolute value for total lymphocytes (P = .02), T lymphocytes (P = .04), absolute CD8 lymphocytes (P = .01) and percentage of CD8 lymphocytes (P = .02) and a higher percentage of CD4 lymphocytes (P = .03) and higher CD4/CD8 ratio (P = .02) than women who had not experienced any pregnancy complications. CONCLUSION: The percentage of B lymphocytes was lower in normal primipara in comparison to women who had never been pregnant. Women with previous spontaneous abortions had an immunologic profile expected in a rejection phenomenon; that result was more marked if they went on to experience complications in their next pregnancies.

Abortion, Spontaneous↗
PubMed1999

[Effect of polycyclic aromatic hydrocarbons on the immune system].

Polycyclic aromatic hydrocarbons (PAHs) are ubiquitous compounds in our environment. They are formed during incomplete burning of coal, oil, gas, wood, garbage or other organic substances such as tobacco and charbroiled meat. PAHs enter the environment mostly as releases to air from volcanoes, forest fires, residential wood and coal burning and exhaust from automobiles and trucks. There are more than 100 different PAHs. Some of them have danger toxic properties including mutagenic and carcinogenic potential. Since 1993 PAHs are classified as the compounds which can cause immunosuppression. They reduce the body resistance against infection and cancer diseases. Immunosuppressive, carcinogenic and hypersensitivity effects of some PAHs representatives were described well in experimental studies. On the other hand, only sporadic information about immunological changes after long-term occupational exposure to PAHs were found from existing human epidemiological database. In addition, these studies usually did not cover the complex immunological profile represented by cellular and humoral activity.

Air Pollutants↗
PubMed2000

Spectrum of renal lesions in HIV patients.

BACKGROUND: A variety of renal lesions have been reported in HIV positive patients from western world however there is paucity of Indian data. METHODS: Over a four year period, all hospitalised HIV positive patients were screened for renal involvement. Screening was done with urinalysis. Those with abnormality in urine examination underwent further assessment with clinical, biochemical, immunological profile and renal biopsy. Renal histology was studied by light and electron microscopy. RESULTS: Twenty-five (17.6%) of the 142 patients screened, had proteinuria/abnormal urinary sediment however none of the patient had proteinuria in nephrotic range. Fourteen of these 25 patients were asymptomatic while others had AIDS. Renal biopsy was studied by light microscopy in all and by electron microscopy in 11 cases. On histology mesangioproliferative GN was encountered in eight, focal segmental glomerulosclerosis in four and collapsing GN in one patient. In two cases cryptococcal infiltration and in one lymphomatous deposits were seen in glomerulus and interstitium. In one patient interstitium showed granulomas and in other three mononuclear cell infiltration. Histology was normal in 8 (32%) patients. On EM visceral cell hyperplasia and vacuolisation was seen in all, two had collapse of glomerular basement membrane and in three cases tubuloreticular structures were seen. There was no co-relation of renal histology with duration or severity of the disease (p > 0.05). No deterioration of renal function was seen over a short follow up period of 4.2 months (1-20 months). CONCLUSION: This study highlights that HIV patients exhibiting abnormal urinary sediment usually have underlying renal lesion and at times unexpected opportunistic infections may be present.

AIDS-Related Opportunistic Infections↗
PubMedDate not supplied

Bronchiectasis in systemic sclerosis. A study using high resolution computed tomography.

OBJECTIVE: To detect noninvasively the presence of bronchiectasis in patients with systemic sclerosis (SSc), through the use of high resolution chest computed tomography (HRCT). METHODS: Twenty two patients with SSc, of whom 13 with diffuse and 9 with limited disease, besides a complete history, physical and routine laboratory and immunologic profile, were evaluated by pulmonary function testing and HRCT. The chi square test with Yates' correction, the Fisher's exact test, the Fisher's test (F test) and the "t" test were used for statistical analysis of the results. RESULTS: Eleven patients (50.0%) had decreased carbon monoxide diffusing lung capacity (DLCO) and, out of these, four had restrictive lung disease, based on a combined decrease of forced vital capacity (FVC) and total lung capacity (TLC). Another two patients exhibited this pattern without DLCO impairment. HRCT revealed a ground glass picture in 15 patients (68.2%), fibrosis in 9 (40.9%) (of which 5 with ground glass as well), and cylindrical bronchiectasis in 13 (59.1%). Bronchiectasis was more common in diffuse than in limited SSc, and the difference approached but did not reach the level of statistical significance. On the other hand, it was not correlated with either decreased DLCO, presence of ground glass and fibrosis, or with patients' age and disease duration. CONCLUSION: Although the number of patients included in our study is relatively small, our data, for the first time in the literature, indicate a significant association between scleroderma and bronchiectasis. Bronchiectasis should be included in the list of pulmonary manifestations of SSc, and SSc in the list of conditions causing bronchiectasis.

Adult↗