Sarcoidosis or primary Sjögren's syndrome?

Correspondence: SIR, We were interested to read the case report by Melsom and coworkers.' We have been followinl up a patient who presented a similar difficult diagnosis

Antibodies to endothelial cells in Behçet's disease: cell-binding heterogeneity and association with clinical activity

OBJECTIVES--To investigate the prevalence and characteristics of antibodies to endothelial cells (aEC) from large vessel and from microvasculature in a group of patients with Behçet's disease (BD) to determine the relationship of these antibodies with clinical and laboratory features of the disease. METHODS--Thirty patients with BD were prospectively and consecutively studied. The aEC were determined by enzyme-linked immunosorbent assay (ELISA) using endothelial cells derived from human umbilical vein (large vessel) as well as from retroperitoneal adipose tissue (microvasculature). RESULTS--Fifteen patients (50%) had aEC, either directed to large vessel [8(26%) patients] or microvascular [13(43%) patients] endothelial cells. The percentage of active patients was significantly higher in the aEC-positive group [12(80%) patients] compared with the aEC-negative group [5(33%) patients] (p < 0.05). CONCLUSIONS--Patients with BD have a high prevalence of aEC when microvascular endothelial cells are used in the assay. These antibodies seem to be a marker of disease activity in this condition, previously considered as negative for autoantibodies

Giant cell arteritis presenting as a supraclavicular nodule

Letters to the editor: Sir: We read with great interest the article by Englert and colleagues on their experiences with sulphasalazine treatment in rheumatoid arthritis (RA).' They noted the disappearance of rheumatoid nodules in four patients with RA in eight to 12 weeks after the start of treatment, parallel with a decrease in disease activity

Pure sensory neuropathy in patients with primary Sjogren's syndrome: clinical, immunological and electromyographic findings

A pure sensory neuropathy caused by lymphocytic infiltration of the dorsal root ganglia has been reported in a few patients with Sjögren's syndrome. The clinical, immunological, and electromyographic findings of five patients with this type of neuropathy and primary Sjögren's syndrome were reviewed. Typical clinical indications were the presence of a chronic asymmetrical sensory deficit, initial disease in the hands with a predominant loss of the vibratory and joint position senses, and an association with Adie's pupil syndrome or trigeminal sensory neuropathy. The simultaneous impairment of the central and peripheral evoked cortical potentials suggested that there was a lesion of the neuronal cell body. The neuropathy preceded the diagnosis of Sjögren's syndrome in four patients. Four patients were positive for Ro antibodies, but systemic vasculitis or malignancy was not found after a mean follow up of six years. These findings indicate that in patients with a sensory neuropathy the diagnosis of Sjögren's syndrome has to be considered, even if the patient denies the presence of sicca symptoms, and that appropriate tests must be carried out

Hepatitis C virus infection in 'primary' Sjögren's syndrome: prevalence and clinical significance in a series of 90 patients

OBJECTIVES—To determine the prevalence and clinical significance of hepatitis C virus (HCV) infection in a large cohort of patients with `primary' Sjögren's syndrome (SS). METHODS—90 consecutive patients (83 female and seven male) were included, with a mean age of 62 years (range 31-80) who prospectively visited our unit. All patients fulfilled the European Community criteria for SS and underwent a complete history, physical examination, as well as biochemical and immunological evaluation for liver disease. Serum from all patients was tested for antibodies to HCV by third generation enzyme linked immunoassay and positivity was confirmed by polymerase chain reaction. RESULTS—Antibodies to HCV were present in 13 (14%) patients with `primary' SS. When compared with patients without HCV infection, patients with HCV infection presented a higher prevalence of hepatic involvement (100% v 8%, p < 0.05). Transcutaneous liver biopsy was performed in five patients with HCV infection, and specimens obtained showed in all cases a chronic active hepatitis with varying degrees of portal inflammation. CONCLUSION—HCV infection is frequent in patients with `primary' SS and liver involvement is present in all these patients. The possible pathogenic role of HCV infection in these patients is still unclear

Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults.

OBJECTIVE—To define the pattern of disease expression in patients with childhood onset systemic lupus erythematosus (SLE). METHODS—Prospective analysis of clinical manifestations and immunological features of 34 patients in whom the first manifestations appeared in childhood from a series of 430 unselected patients with SLE. RESULTS—Thirty one (91%) patients from the childhood onset group were female and three male (9%) (ratio female/male, 10/1, with no difference compared with the adult onset group). Mean age of this group at disease onset was 11 years (range 5-14) compared with 32 years (15-48) for the remaining patients. The childhood onset patients more often had nephropathy (20% v 9% in adult onset SLE, p=0.04; OR:2.7; 95%CI:1.1, 7), fever (41% v 21%, p=0.006; OR:2.6, 95%CI:1.2, 5.7), and lymphadenopathy (6% v 0.5%, p=0.03, OR: 12.3, 95%CI: 1.2, 127.6), as presenting clinical manifestations. During the evolution of the disease, the childhood onset patients had an increased prevalence of malar rash (79% v 51%, p=0.002; OR:3.7; 95%CI:1.5, 9.5) and chorea (9% v 0%, p<0.0001). This group exhibited a higher prevalence of anticardiolipin antibodies (aCL) of the IgG isotype when compared with the remaining patients (29% v 13%, p=0.017; OR:2.9, 95%CI:1.2, 6.8). No significant differences were found among the other antibodies between the two groups. Childhood onset patients more often received azathioprine (15% v 6%, p=0.00004; OR:11.2; 95%CI:2.8, 44.9) but no differences were detected between the groups concerning side effects or drug toxicity. CONCLUSIONS—The presentation and the clinical course of SLE varied in this series of 430 patients depending on their age at disease onset. Nephropathy, fever, and lymphadenopathy were more common in childhood onset patients as presenting clinical manifestations, while malar rash, chorea, and detection of IgG aCL were more common during the evolution of the disease

Systemic lupus erythematosus in men: clinical and immunological characteristics.

Although systemic lupus erythematosus (SLE) has traditionally been considered a disease of women, men may also be affected. Thirty of 261 patients (12%) with SLE seen in this hospital were men. Arthritis was less common as a first symptom in the men, although this group of patients had discoid lesions and serositis more often than the women. During the follow up a lower incidence of arthritis and malar rash and a higher incidence of other skin complications including discoid lesions and subcutaneous lupus erythematosus was found in the men. The incidence of nephropathy, neurological disease, thrombocytopenia, vasculitis, and serositis, was similar in the two groups. No significant immunological differences were found between men and women. These features indicate that several gender associated clinical differences may be present in patients with SLE

Systemic lupus erythematosus without clinical renal abnormalities

SIR, We read with interest the study of O'Dell et all showing a high incidence of mesangial changes in renal biopsies from patients with systemic lupus erythematosus (SLE) without clinical renal abnormalities. They found no cases of focal or diffuse proliferative glomerulonephritis. These results are different from those previously reported by other authors2 3 and question the relevance of renal biopsy in these patients..

Hepatitis G virus infection in primary Sjögren's syndrome: analysis in a series of 100 patients.

OBJECTIVE To determine the prevalence and clinical significance of hepatitis G virus (HGV) infection in a large cohort of patients with primary Sjögren¿s syndrome (SS). PATIENTS AND METHODS The study included 100 consecutive patients (92 female and eight male), with a mean age of 62 years (range 31¿80) that were prospectively visited in our unit. All patients fulfilled the European Community criteria for SS and underwent a complete history, physical examination, as well as biochemical and immunological evaluation for liver disease. Two hundred volunteer blood donors were also studied. The presence of HGV-RNA was investigated in the serum of all patients and donors. Aditionally, HBsAg and antibodies to hepatitis C virus were determined. RESULTS Four patients (4%) and six volunteer blood donors (3%) presented HGV-RNA sequences in serum. HGV infection was associated with biochemical signs of liver involvement in two (50%) patients. When compared with primary SS patients without HGV infection, no significant differences were found in terms of clinical or immunological features. HCV coinfection occurs in one (25%) of the four patients with HGV infection. CONCLUSION The prevalence of HGV infection in patients with primary SS is low in the geographical area of the study and HCV coinfection is very uncommon. HGV infection alone does not seen to be an important cause of chronic liver injury in the patients with primary SS in this area

Splenectomy for refractory Evan' syndrome associated with antiphospholipid antibodies: report of two cases.

The main haematological manifestations seen in patients with antiphospholipid antibodies (aPL) are thrombocytopenia, usually mild, and haemolytic anaemia with a positive Coombs test. Owing to the shared characteristics with idiopathic thrombocytopenic purpura, similar rules are followed in the treatment of these cytopenias. Two patients with severe aPL associated cytopenias, who required splenectomy after being refractory to steroids, immunosuppressive agents, and other treatments (intravenous gammaglobulin, danazol), are described, and previously reported cases are reviewed