Anticardiolipin (aCL) antibodies in a patient with Behçet's syndrome with vena cava inferior thrombosis.

This case report describes a patient with Behçet's syndrome with sustained anticardiolipin antibodies presenting a vena cava inferior thrombosis in a period of anabolic steroid use. The vascular manifestations and occurrence of antiphospholipid antibodies in Behçet's syndrome are reviewed. The possible thrombogenicity of anabolic steroids is reemphasized.

The clinical manifestations of ochronosis: a review.

Ochronosis is a rare disease caused by an inherited lack of homogentisic acid oxidase. Alkaptonuria is the presence of homogentisic acid in urine. Ochronosis is characterized by the deposition of a dark pigment in tissues rich in collagen. A wide spectrum of clinical manifestations is described. The most important signs are ochronotic arthropathy, ocular and cutaneous pigmentation, genitourinary tract obstruction by ochronotic calculi and cardiovascular ochronosis, especially calcification and stenosis of the aortic valve. The constellation of these clinical signs should suggest the diagnosis which is confirmed by the detection of homogentisic acid in urine. There is no cure for the disease and treatment is based on symptomatic measures.

Ultrasonography of the salivary glands in the evaluation of Sjögren's syndrome. Comparison with sialography.

Ultrasonography (US) was evaluated as noninvasive method to determine salivary gland involvement and compared with sialography in patients with Sjögren's syndrome (SS) and patients with only objective xerostomia and/or xerophthalmia. Patients with complaints of dry eyes and dry mouth but with no objective abnormalities served as control group. Ultrasound revealed a decreased parotid gland reflectivity in most of the patients with SS, compared with only 2 patients in the other group (p = 0.002). Our study suggests that US can be a useful, noninvasive method in the diagnostic work-up of patients with sicca complaints.

D-penicillamine induced pemphigus treated with methylprednisolone pulse therapy.

A patient with severe rheumatoid arthritis received d-penicillamine treatment for 16 months. She developed mild pruritus 6 months after d-penicillamine initiation, and severe pemphigus 10 months after d-penicillamine was changed to azathioprine. Subsequent methylprednisolone pulse therapy resulted in a transient clinical remission of pemphigus. A literature review of d-penicillamine induced pemphigus and high dose methylprednisolone pulse therapy is presented.

Humoral immunity and composition of immune complexes in patients with rheumatoid arthritis, with special reference to IgE-containing immune complexes.

IgE-containing circulating immune complexes (IgE-CIC) were determined with a 2.5% PEG-precipitation assay in 98 patients with classical or definite rheumatoid arthritis (RA). Of the 45 IgE-CIC positive sera, only 4 had elevated total serum IgE. IgE-CIC positive patients had more active disease than patients without IgE-CIC, as determined by their more swollen joints and higher Ritchie indices (p less than 0.04 and 0.02, respectively). Apart from IgE, other immunoglobulin isotypes, rheumatoid factor (RF) of the IgG-, IgA- and IgM-classes, C3 and antinuclear antibodies could be demonstrated in the IgE-containing PEG-precipitates. IgE-RF could not be demonstrated in serum or in IgE-CIC. Anti-IgE of the IgM-class (IgMaIgE) were frequently found (28/45 patients) in the IgE-positive PEG-precipitates. All 14 patients positive for IgGaIgE in the IgE-CIC were also positive for IgMaIgE in the CIC. As in the serum, there was a good correlation in the CIC between the level of IgGaIgE and the level of IgMaIgE (r = 0.64). The correlation between the respective levels of IgGaIgE and IgMaIgE in serum and in CIC was high (r = 0.93 and 0.79, respectively). On the other hand, only 1 patient was positive for IgAaIgE in the IgE-CIC. We conclude that IgE and aIgE of the IgM- and IgG-classes are frequently present in the immune complex form in RA and that they are correlated with the clinical activity of arthritis.

Ultrasonography and computer tomography of the salivary glands in the evaluation of Sjögren's syndrome. Comparison with parotid sialography.

Ultrasonography (US) and computed tomography (CT) were studied as noninvasive methods to determine salivary gland involvement and compared with parotid sialography in patients with Sjögren's syndrome (SS) and patients with only objective xerostomia and/or xerophthalmia. Patients with complaints of dry eyes and dry mouth but with no objective abnormalities served as controls. CT was not discriminative between the 2 groups. In contrast, US revealed decreased parotid gland echogenicity in most of the patients with SS, compared with only 1 patient in the group with sicca complaints only (p = 0.005). Our study suggests that US can be a useful, noninvasive method in the diagnostic investigation of patients with sicca complaints.

D-penicillamine therapy and interstitial lung disease in scleroderma. A long-term followup study.

Sequential lung function tests were performed on 17 scleroderma patients who were treated with D-penicillamine (DP) (total of 66 treatment years) and on 10 control scleroderma patients who were not treated or were treated with low-dose prednisone (total of 25 treatment years). Cusum plots showed significant differences between the 2 groups in their cumulative changes in carbon monoxide diffusing capacity (DLCO) (P less than 0.005) and in DLCO/lung volume (P less than 0.02). The end value of the DLCO was greater than 10% lower than the initial value in 3 of the 17 DP-treated patients versus 5 of the 10 control patients (P less than 0.01, Fisher's exact probability test); in 3 DP-treated patients and 8 control patients (P less than 0.003, Fisher's exact probability test), the end value of the DLCO/lung volume was greater than 10% lower than the initial value. We conclude that DP has a beneficial effect on interstitial lung disease in patients with scleroderma.