Joaquin Coll

Assessment of the European classification criteria for Sjögren's syndrome in a series of clinically defined cases: results of a prospective multicentre study. The European Study Group on Diagnostic Criteria for Sjögren's Syndrome.

OBJECTIVE: To assess the recently proposed preliminary criteria for the classification of Sjögrens syndrome (SS) in a multicentre European study of a new series of clinically defined cases. METHODS: The criteria included six items: I = ocular symptoms; II = oral symptoms; III = evidence of keratoconjunctivitis sicca; IV = focal sialoadenitis by minor salivary gland biopsy; V = instrumental evidence of salivary gland involvement; VI = presence of autoantibodies. Each centre was asked to provide five patients with primary SS, five with secondary SS, five with connective tissue diseases (CTD) but without SS, and five controls (patients with ocular or oral features that may simulate SS). The preliminary six item classification criteria set was applied to both the SS patients and the non-SS controls, and the performance of the criteria in terms of sensitivity and specificity was tested. RESULTS: The criteria set was tested on a total of 278 cases (157 SS patients and 121 non-SS controls) collected from 16 centres in 10 countries. At least four of the six items in the criteria set (limiting item VI to the presence of Ro(SS-A) or La(SS-B) antibodies) were present in 79 of 81 patients initially classified as having primary SS (sensitivity 97.5%), but in only seven of 121 non-SS controls (specificity 94.2%). When the presence of item I or II plus any two of items III-V of the criteria set was considered as indicative of secondary SS, 97.3% (71 of 73) of the patients initially defined as having this disorder and 91.8% (45 of 49) of the control patients with CTD without SS were correctly classified. CONCLUSION: This prospective study confirmed the high validity and reliability of the classification criteria for SS recently proposed by the European Community Study Group.

Bone remodelling in human immunodeficiency virus-1-infected patients. A histomorphometric study

The aim of this study was to identify and describe possible alterations of bone histomorphometry in patients with human immunodeficiency virus (HIV-1) infection and to assess the relation between these alterations and disease severity. Forty-four HIV-1-infected patients seen successively at our hospital were evaluated for the study. In an attempt to avoid confounding factors as far as possible, we excluded patients who fulfilled any of the following criteria: age less than 18 or greater than 40 years; recent history of extended bed rest; previous diagnosis of metabolic bone disease, renal insufficiency, or hepatic failure; clinical or echographic signs of liver cirrhosis; diabetes mellitus or previous diagnosis of other endocrine diseases; drug therapy that could act on bone metabolism; and/or moderate to severe nutritional alteration. Twenty-two patients (13 men, 9 women; age: 27.9 +/- 4.1 years, mean +/- standard deviation) were included in the study. Plasma and urine biochemistry and calcium-regulating hormones were determined. Bone mineral content was measured on vertebrae L2 to L4 and on the neck and intertrochanteric areas of the femur by dual-photon absorptiometry. A transiliac bone biopsy was performed after double-tetracycline labelling, with histomorphometric study of undecalcified bone. Serum osteocalcin was found to be lower in patients who, according to the Centers for Disease Control (CDC) classification, had greater disease severity, and showed a positive correlation with the number of CD4+ T lymphocytes. No alterations in bone densitometry were observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Immunohistochemistry of minor salivary gland biopsy specimens from patients with Sjögren’s syndrome with and without hepatitis C virus infection

OBJECTIVES To characterise phenotypically the minor salivary glands of patients with clinical and histological features of Sjögren’s syndrome (SS) infected with hepatitis C virus (HCV). PATIENTS AND METHODS 75 consecutive patients with SS (31 primary SS, 44 secondary SS) diagnosed by preliminary European classification criteria. The presence of anti-HCV antibodies was detected by commercial third generation ELISA and by a second generation immunoblot assay. Presence of HCV genome in serum was determined by polymerase chain reaction analysis. Expression of CD3, CD4, CD8, CD20, HLA-DR, and CD25 molecules in lymphocytic and epithelial cells on minor salivary glands was detected by immunohistochemical assays. Expression of interferon γ and interleukin 4 cytokines was determined by in situ hybridisation. RESULTS Six of 31 primary SS (19%) and one of 44 secondary SS (2%) serum samples were positive for anti-HCV by ELISA. Three samples were positive, three indeterminate, and one sample corresponding to a secondary SS patient was negative by immunoblot. The three immunoblot positive serum samples were also HCV-RNA positive by PCR assay. The study of lymphocytic cells in the diffuse infiltrate of minor salivary glands showed a predominance of the CD3 lymphocytic population. A predominance of CD4 over CD8 T cells (ratio 2:1) was observed in HCV and non- HCV infected patients. The analysis of the lymphocytic focus showed that the HCV infected patients had a predominance of CD20 positive cells. Activation molecules ( CD-25 and HLA-DR ) were expressed in HCV and non-HCV infected patients in lymphocytic and epithelial cells, however epithelial cell expression of CD25 was low in HCV infected patients. As expected, a pronounced Th1 response was observed in the lymphocytic foci of HCV patients. CONCLUSIONS HCV infected patients may develop an autoimmune sialadenitis, similar to that described in primary SS.

Pulmonary tuberculosis in HIV-infected patients with normal chest radiographs

SubjectsThree HIV-infected patients with active pulmonary non-disseminated tuberculosis and normal chest radiograph at clinical presentation and during follow-up are reported. Patients had cough and fever but no other specific symptoms. Löwenstein cultures of specimens from bronchoalveolar lavage in two cases and induced sputum in one yielded Mycobacterium tuberculosis. ConclusionsThe diagnosis of tuberculosis in HIV-infected patients depends greatly on clinical suspicion by the physician, because of its atypical presentation. Failure to perform appropriate diagnostic tests in HIV-infected patients who present with suspected pulmonary disease will result in underdiagnosis and undertreatment of tuberculosis.

Sjögren's syndrome: a stepwise approach to the use of diagnostic tests.

One hundred and forty two patients (62 with definite Sjögrens syndrome, 24 with probable Sjögrens syndrome, and 56 in whom Sjögrens syndrome was finally ruled out) were studied. Schirmers test and rose bengal staining for the diagnosis of keratoconjunctivitis sicca and salivary scintigraphy and a labial biopsy sample for the diagnosis of xerostomaia were studied in all patients. Rose bengal staining showed high specificity (98%) but low sensitivity (55%). All patients with positive rose bengal staining results had associated xerostomia. In the rose bengal staining positive patients, scintigraphy had 100% specificity. A labial biopsy sample showed high sensitivity in the rose bengal staining, salivary scintigraphy positive group, and high specificity in the rose bengal staining positive, salivary scintigraphy negative group. In patients with negative rose bengal staining, salivary scintigraphy showed 96% specificity and 36% sensitivity. A labial biopsy sample had a sensitivity and specificity greater than 90% in rose bengal staining negative patients. Only 29 biopsy samples were needed to achieve a diagnosis of Sjögrens syndrome in 142 patients (20%). Hence the suggested approach may make it unnecessary to take biopsy samples in approximately 80% of patients with suspected Sjögrens syndrome. Using the stepwise approach of first rose bengal staining, then salivary scintigraphy, and eventually a labial biopsy sample in patients with suspected Sjögrens syndrome, the diagnosis is relatively simple.

Radiographic findings in pulmonary tuberculosis : the influence of human immunodeficiency virus infection

The chest radiographs and medical records of 166 patients diagnosed as having clinically active pulmonary tuberculosis were reviewed. Forty-nine patients (group I) were seropositives to human immunodeficiency virus (HIV), and 117 patients (group II) did not have known risk factors for HIV infection. Roentgenographic abnormalities were analysed in the two groups, according to nine different radiographic patterns previously defined. The seropositive group had a significantly higher proportion of hilar and/or mediastinal adenopathy (P less than 0.001), infiltrates confined to the lower lung fields (P less than 0.05), and miliary tuberculosis (P less than 0.005). Otherwise, single cavitation and destructive pattern were more frequent in the group II. These data suggest that patients with pulmonary tuberculosis and HIV infection are much more likely to have atypical radiographic findings.

Lacrimal Immunoglobulins in Rheumatoid Arthritis Patients with or without Sjögren’s Syndrome

The aim of the present study was to analyse immunoglobulin G, A, and M levels in tears of patients with rheumatoid arthritis with or without keratoconjunctivitis sicca (KS) which define Sjögren’s syndrome (SS). Tears were collected from the lower cul-de-sac by capillarity (100–300 μl). Tear IgG, IgA and IgM levels were determined by radial immunodiffusion. Samples diluted 1:10 were used for IgA and IgG and non-diluted ones for IgM. Fifty-three patients with rheumatoid arthritis and 30 healthy control subjects were studied. In all individuals IgA predominated in tears, IgG levels were low but with a very wide range and IgM was present in very low concentrations. The IgG concentration in tears showed statistically significant differences between the control group and that of rheumatoid arthritis and KS. IgG in tears correlated positively with the rose bengal test (r = 0.2848, p < 0.05) and negatively with the Schirmer test (r = –0.3042, p < 0.05). Tear IgG measurement might provide a marker for eye involvement in patients with rheumatoid arthritis and KS which define SS.

Tuberculous pericarditis as the first manifestation of AIDS

Although it is known that extrapulmonary tuberculosis in patients with AIDS has a broad spectrum of clinical manifestations, pericardial involvement has rarely been described. We report a case of this condition as the first manifestation of AIDS with good response to antituberculous therapy. Although unusual, the diagnosis of tuberculous pericarditis in symptomatic HIV-infected patients should be aggressively pursued because of the beneficial clinical response to treatment. Bei AIDS-Patienten sind zahlreiche Manifestationsformen der extrapulmonalen Tuberkulose bekannt; eine Perikardbeteiligung wurde jedoch nur selten beschrieben. Wir berichten über einen Fall von tuberkulöser Perikarditis bei einem AIDS-Patienten mit gutem Ansprechen auf die antituberkulöse Therapie. Trotz der Seltenheit einer tuberkulösen Perikarditis bei Patienten mit symptomatischer HIV-Infektion sollte wegen der günstigen Behandlungsmöglichkeiten bei Verdacht eine aggressive Diagnostik durchgeführt werden.SummaryAlthough it is known that extrapulmonary tuberculosis in patients with AIDS has a broad spectrum of clinical manifestations, pericardial involvement has rarely been described. We report a case of this condition as the first manifestation of AIDS with good response to antituberculous therapy. Although unusual, the diagnosis of tuberculous pericarditis in symptomatic HIV-infected patients should be aggressively pursued because of the beneficial clinical response to treatment.ZusammenfassungBei AIDS-Patienten sind zahlreiche Manifestationsformen der extrapulmonalen Tuberkulose bekannt; eine Perikardbeteiligung wurde jedoch nur selten beschrieben. Wir berichten über einen Fall von tuberkulöser Perikarditis bei einem AIDS-Patienten mit gutem Ansprechen auf die antituberkulöse Therapie. Trotz der Seltenheit einer tuberkulösen Perikarditis bei Patienten mit symptomatischer HIV-Infektion sollte wegen der günstigen Behandlungsmöglichkeiten bei Verdacht eine aggressive Diagnostik durchgeführt werden.