Serum C4 was 0.32 g/l (RR 0.16 to 0.58 g/l). X-ray of the hands showed acro-osteolysis of the terminal phalange of thumb and index finger. the best characterized renal involvement in scleroderma is usually scleroderma renal crisis. However, other renal pathologies can occur in scleroderma. These alternative pathologies should be suspected in any patient with a differing clinical picture and Evista (Raloxifene HCl) the patient should be appropriately investigated, as the clinical course and treatment are different from the more common scleroderma renal crisis. Introduction Scleroderma (systemic sclerosis) is a chronic systemic disease that targets the skin, lungs, heart, gastrointestinal tract, kidneys and musculoskeletal system. The disorder is usually characterized by three features: tissue Evista (Raloxifene HCl) fibrosis, small blood vessel vasculopathy and a specific autoimmune response associated with autoantibodies. Scleroderma is usually classified into two major subsets, diffuse and limited cutaneous sclerodermas, that are distinguished by the extent of skin thickening. Diffuse scleroderma is usually characterized by widespread skin thickening involving distal and proximal body regions; rapid onset (within 1 year) of skin Tshr and other features following appearance of Raynaud’s phenomenon; significant visceral involvement; high scores on disability and organ damage indices secondary to extensive fibrosis of tissues associated with antinuclear antibodies; and the absence of anticentromere antibody. Limited scleroderma shows limited skin thickening, slow progression of disease and late visceral involvement, with unique features of isolated pulmonary hypertension and digital amputations associated with anticentromere antibody. Overlap syndromes have diffuse or limited scleroderma features plus features common of one or more other connective tissue or autoimmune diseases. Mixed connective tissue disease shows features of scleroderma, systemic lupus erythematosus polymyositis, rheumatoid arthritis and the presence of anti-U1 sn-RNP antibodies. Approximately 10% of patients with scleroderma have a renal crisis that mimics malignant hypertension, with rapidly progressive renal failure secondary to microvascular disease, vasospasm and tissue ischemia. Microangiopathic hemolytic anemia and thrombocytopenia can accompany scleroderma renal crisis. Studies demonstrate high levels of serum renin levels associated with vasospasm and intrinsic renal vessel disease. A renal crisis is usually associated with the use of corticosteroids or can be precipitated by conditions compromising renal blood flow (dehydration). Any hypertension ( 140/90 mmHg) in a scleroderma patient should be carefully evaluated because a renal crisis is usually potentially reversible with appropriate management with angiotensin converting enzyme Evista (Raloxifene HCl) (ACE) inhibitors. Patients presenting with serum creatinine above 270 mol/l have a poor prognosis. Some patients who progress to renal failure and dialysis can recover renal function after months of dialysis therapy. Variable changes may be seen in the glomeruli. In some cases thickening of glomerular capillary walls with a double contour appearance on silver or periodic acid-Schiff staining may be seen. Fibrinoid necrosis may also be seen. Crescents are very rare and those that are seen are invariably small. Interlobular arteries show intimal thickening which is mucinous or finely fibrous. The thickening results in a considerable reduction of the lumen. Crescentic glomerulonephritis (GN) represents a severe form of glomerular disease that is characterized by disruption of the glomerular basement membrane, leading to cellular proliferation in the Bowman’s space and is often accompanied by fibrinoid necrosis. Crescentic GN is usually classified into three major types. Anti-glomerular basement membrane (anti-GBM) disease is usually characterized by circulating anti-GBM antibodies and linear deposition of antibodies along the glomerular basement membrane. This constitutes around 10% of cases. Pauci-immune (anti-neutrophil cytoplasmic antibodies (ANCA)-associated GN) is usually characterized by scanty glomerular deposits of immunoglobulin and circulating ANCA, and comprises about 60% of cases. Immune complex-mediated GN is a heterogeneous group of diseases usually associated with obvious granular deposits of immunoglobulins, in which crescent formation complicates.