Thrombolytic treatment in a patient with antiphospholipid syndrome : APS developing renal infarction.

Antiphospholipid syndrome (APS), a leading entity in acquired thrombophilia, is characterized by recurrent thrombosis, morbidity in pregnancy and presence of antiphospholipid antibodies (APA). Although the etiopathogenesis is unclear, APA against negatively charged phospholipids and phospholipid-protein complexes are held responsible for the clinical picture. In case of acute thrombosis due to APS, thrombolytic therapy is not a commonly administered treatment option. Here, we present a case with acute thrombosis in the left renal artery showing partial response to thrombolytic therapy.

A case of Amyopathic Dermatomyositis associated with Interstitial Pulmonary Disease.

Inflammatory myopathies are a heterogeneous group of diseases with unknown etiology characterized by inflammation of the skeletal muscles and proximal muscle weakness. Dermatomyositis (DM) is an idiopathic inflammatory myopathy with characteristic cutaneous findings such as heliotrope rash, Gottron's sign, Gottron's papules, shawl sign and machinist hand. Amyopathic dermatomyositis (ADM) is a rare but well-recognized clinical subtype of DM, constituting aproximately 10-20% of patients with this disease. It generally manifests only pathognomonic skin findings without clinical and laboratory evidence of muscle involvement. In this report, we present a rare case of ADM associated with interstitial pulmonary disease.

Nail changes in connective tissue diseases: do nail changes provide clues for the diagnosis?

OBJECTIVE: To evaluate the frequency and the specificity of nail changes associated with connective tissue diseases (CTD). METHODS: In a case-control study, 190 patients including those with systemic lupus erythematosus (SLE; 56), rheumatoid arthritis (RA, 47), primary Sjögren's syndrome (pSS; 35), systemic sclerosis (SSc; 39), and dermatomyositis/polymyositis (DM/PM; 13) were enrolled in the study. Patients with SLE and other CTDs were compared with two different control groups. Twenty nails were examined. Nail features were noted and classified. Nail samples were collected for mycological cultures. RESULTS: In patients with SLE, erythema of proximal nailfold (P<0.01), splinter haemorrhages in fingernails (P<0.01), capillary loops in proximal nailfold (P<0.05), periungual erythema (P<0.05), and thin nail plates (P<0.05) were more common than those in controls. Only splinter haemorrhages were associated with the disease activity. In patients with SSc and DM/PM, splinter haemorrhages (P<0.05) and capillary loops in proximal nailfold (P<0.01) in fingernails were common as well. Increase in longitudinal curvature (P<0.001), transverse curvature (P<0.01), and white dull colour in fingernails were other frequent findings in patients with SSc. Increase in transverse curvature was associated with the disease activity in SSc. In patients with RA, splinter haemorrhages (P<0.05), red lunula (P<0.05), and white dull colour (P<0.05) in fingernails were frequent. The sensitivity values of all these changes were very low. However, their specificity values were found to be relatively high. CONCLUSION: Proximal nailfold is the most important site of affection in CTDs. These nail changes can be used in combination with highly sensitive diagnostic modalities to establish an accurate diagnosis.

Serum soluble Fas levels in patients with autoimmune rheumatic diseases.

OBJECTIVE: The role of the serum soluble Fas (sFAS) system is unclear in diagnosis of several autoimmune rheumatic diseases although there are present contradictory reports on the levels of serum sFas. We therefore assessed levels of sFAS in serum of patients with autoimmune rheumatic diseases. PATIENTS AND METHODS: We analyzed sFas levels and their relationship to clinical and laboratory data in patients with systemic lupus erythematosus (SLE, n=32), rheumatoid arthritis (RA, n=28), Sjögren's syndrome (SS, n=20) systemic sclerosis (SSc, n=21), polymyositis/dermatomyositis (PM/DM, n=15). Patients with osteoarthritis (OA, n=20) and healthy volunteers (n=20) were used as controls. Serum levels of sFAS were determined by ELISA. sFas levels greater than mean (normals)+2 SD were considered as elevated. RESULTS: The mean sFas values were found higher in RA, PM/DM and OA than in control although no differences were found in SSc and SS patients. The mean sFas levels in SLE patients were lower than healthy controls. Elevated sFas rates in RA, PM/DM and SS were found to be 21.4%, 60%, 10% higher than in healthy controls, respectively. sFas levels in SLE and SSc did not differ from control values. Mean sFas levels did not show significant difference between active and inactive patients in all disease groups except PM/DM, RA and OA. No correlations of sFas with relevant disease subsets, laboratory findings and treatment modalities were found. CONCLUSIONS: The findings indicate that the serum sFas molecule may provide a useful additional marker for presence and assessment of disease in patients with RA and PM/DM.