No cure for<unk>  is known, although relief of symptoms is often achieved; these include the: * Raynaud's phenomenon with vasodilators such as calcium channel blockers, alpha blockers, serotonin receptor antagonists, angiotensin II receptor inhibitors, statins, local nitrates or iloprost * Digital ulcers with phosphodiesterase 5 inhibitors (e.g., sildenafil) or iloprost * Prevention of new digital ulcers with bosentan * Malnutrition, secondary to intestinal flora overgrowth with tetracycline antibiotics such as tetracycline * Interstitial lung disease with cyclophosphamide, azathioprine with or without corticosteroids * Pulmonary arterial hypertension with endothelin receptor antagonists, phosphodiesterase 5 inhibitors, and prostanoids * Gastrooesophageal reflux disease with antacids or prokinetics * Kidney crises with angiotensin converting enzyme inhibitors and angiotensin II receptor antagonists Systemic disease-modifying treatment with immunosuppressants is often used. Immunosuppressants used in its treatment include azathioprine, methotrexate, cyclophosphamide, mycophenolate, intravenous immunoglobulin, rituximab, sirolimus, alefacept, and the tyrosine kinase inhibitors, imatinib, nilotinib, and dasatinib. Experimental therapies under investigation include endothelin receptor antagonists, tyrosine kinase inhibitors, beta-glycan peptides, halofuginone, basiliximab, alemtuzumab, abatacept, and haematopoietic stem cell transplantation. Immunomodulatory agents in the treatment of<unk>  INN Mechanism of action Route of administration Pregnancy category Major toxicities Alefacept Monoclonal antibody to inhibit T lymphocyte activation by binding to CD2 portion of human leukocyte function antigen-3. IM B (US) Malignancies, injection site reactions, blood clots, lymphopenia, hepatotoxicity and infections. Azathioprine Purine analogue that inhibits lymphocyte proliferation via conversion to mercaptopurine PO, IV D (Au) Myelosuppression and rarely malignancy, hepatitis, infection, hepatic sinusoidal obstruction syndrome and hypersensitivity reactions. Cyclophosphamide Nitrogen mustard that cross-links DNA base pairs, leading to breakages and triggering apoptosis in haematopoietic cells. PO, IV D (Au) Vomiting, myelosuppression, haemorrhagic cystitis and rarely heart failure, pulmonary fibrosis, hepatic sinusoidal obstruction syndrome, malignancy and SIADH Dasatinib Tyrosine kinase inhibitor against various proangiogenic growth factors (including PDGF and VEGF). PO D (Au) Fluid retention, myelosuppression, haemorrhage, infections, pulmonary hypertension, electrolyte anomalies and uncommonly hepatotoxicity, heart dysfunction/failure, myocardial infarction, QT interval prolongation, renal failure and hypersensitivity. Imatinib As above PO D (Au) As above and rarely: GI perforation, avascular necrosis and rhabdomyolysis Immunoglobulin Immunoglobulin, modulates the immune system. IV N/A Varies Methotrexate Antifolate; inhibits dihydrofolate reductase. PO, IV, IM, SC, IT D (Au) Myeosuppression, pulmonary toxicity, hepatotoxicity, neurotoxicity and rarely kidney failure, hypersensitivity reactions, skin and bone necrosis, and osteoporosis Mycophenolate Inosine monophosphate dehydrogenase inhibitor, leading to reduced purine biosynthesis in lymphocytes. PO, IV D (Au) Myelosuppression, blood clots, less commonly GI perforation/haemorrhage and rarely pancreatitis, hepatitis, aplastic anaemia and pure red cell aplasia. Nilotinib As per dasatinib PO D (Au) As per imatinib Rituximab Monoclonal antibody against CD20, which is expressed on B lymphocytes IV C (Au) Infusion-related reactions, infection, neutropenia, reduced immunoglobulin levels, arrhythmias, less commonly anaemia, thrombocytopenia, angina, myocardial infarction, heart failure, and rarely haemolytic anaemia, aplastic anaemia, serum sickness, severe skin conditions, pulmonary infiltrates, pneumonitis, cranial neuropathy (vision or hearing loss) and progressive multifocal leucoencephalopathy. Sirolimus mTOR inhibitor, thereby reducing cytokine-induced lymphocyte proliferation. PO C (Au) Neutropenia, hypokalaemia, interstitial lung disease, pericardial effusion, pleural effusion, less commonly pulmonary haemorrhage, nephrotic syndrome, and rarely hepatotoxicity and lymphoedema. PO = Oral. IV = Intravenous. IM = Intramuscular. SC = Subcutaneous. IT = Intrathecal. The preferred pregnancy category, above, is Australian, if available. If unavailable, an American one is substituted.
Scleroderma
Mixed connective tissue disease
Chyloderma
Dermatomyositis
Anetoderma
Polymyositis
Xeroderma
Lupus