Cm Black

Role of thrombin in pulmonary fibrosis

Pulmonary fibrosis commonly develops in systemic sclerosis. We assessed the role of thrombin in promoting fibroblast proliferation in the lungs in this disorder. Bronchoalveolar lavage fluid (BALF) thrombin concentrations were higher in ten patients with systemic sclerosis than in 12 healthy controls (14.6 vs 3.6 nmol/L, p < 0.02), but values in patients with cryptogenic fibrosing alveolitis (n = 10) or sarcoidosis (n = 10) were not increased. BALF from all patients induced fibroblast proliferation. This proliferation was attenuated by thrombin inhibitors for BALF from systemic sclerosis patients only. We suggest thrombin contributes to lung fibroblast proliferation in this disorder.

Systemic sclerosis: current pathogenetic concepts and future prospects for targeted therapy

Although there is no cure for systemic sclerosis, the disease is certainly treatable. And the more that is understood of its pathogenesis, the better the prospects for treatment. Excessive collagen deposition in the skin and viscera is responsible for the morbidity and mortality. The immune system plays a critical role in setting up the fibrotic process, but impaired regulation of collagen gene expression probably underlies the persistence of fibrosis. The development of effective antifibrotic drugs, in particular agents that inhibit transcription factors involved in the activation of type I collagen genes, is likely to be the way forward for treatment of the later stages of diffuse systemic sclerosis, with immunomodulatory therapy for the earlier stages.

Cyclical psoriatic arthritis responding to anti‐oestrogen therapy

We report a patient with severe psoriatic arthritis in whom the severity of both the arthritis and psoriasis fluctuated with the menstrual cycle. These features failed to improve with standard therapy, but there was a prompt response to treatment which suppressed oestrogen secretion. Such treatment should be considered in patients with disabling cyclical changes in psoriatic arthritis.

Generalized morphoea secondary to porphyria cutanea tarda

We report a patient with generalized morphoea secondary to porphyria cutanea tarda. who responded to treatment with cyclosporin. The differences between the sclerodermatous skin changes seen in porphyria cutanea tarda and generalized morphoea are discussed.

Atypical systemic sclerosis following exposure to vinyl chloride monomer. A case report and review of the cutaneous aspects of vinyl chloride disease

An unusual case of systemic sclerosis occurring in a patient exposed to the vinyl chloride monomer (VCM) is presented. The dermatological aspects of vinyl chloride disease (VCD) are outlined and the mechanisms of pathogenesis discussed.