| Antiphospholipid syndrome | Multiple and recurrent venous and arterial thromboses, recurrent abortions. Secondary form is associated with systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, and Sjögren's syndrome. Thrombophlebitis and deep vein thrombosis, thrombocytopenia, hemolytic anemia, microangiopathic hemolytic anemia, livedo reticularis, stroke, transient ischemic attack, eye vascular complications. Coronary, renal, mesenteric, and cerebral vascular occlusion. | ARDS, pulmonary embolism, ischemic complications of vascular occlusion, bleeding, severe anemia, vision loss, catastrophic antiphospholipid syndrome. |
| Ankylosing spondylitis | Chronic inflammatory disease of the axial skeleton, with progressive stiffness of the spine. Young adults (peak at 20 and 30 years old). Back pain (improves with exercise), buttock, hip, or shoulder pain, systemic complaints (fever, malaise, fatigue, weight loss, myalgias), uveitis, and restrictive pulmonary failure due to costovertebral rigidity, ILD, renal impairment, fracture of the ankylosed spine, asymptomatic ileal and colonic mucosal ulcerations. Secondary amyloidosis. | Acute spinal cord or nerve compression, subluxation of the atlantoaxial joint, aortic regurgitation. |
| Adult Still's disease | Inflammatory disorder (similar to systemic onset juvenile rheumatoid arthritis). Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), arthritis, myalgia, evanescent rash, pharyngitis, lymphadenopathy, splenomegaly, anemia, thrombocytopenia. Pericarditis, myocarditis, pleurisy. | ARDS, arrhythmias, heart failure, fulminant hepatic failure, red cell aplasia, disseminated intravascular coagulation, microangiopathic hemolytic anemia. |
| Behçet's disease | Chronic, relapsing, inflammatory disease. Systemic vasculitis involving arteries and veins of all sizes (carotid, pulmonary, aortic, and inferior extremity vessels are most commonly involved, with aneurysm, dissection, rupture, or thrombosis). Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), recurrent painful skin and mucosal lesions; asymmetric, nondeforming arthritis of the medium and large joints; thrombophlebitis and deep vein thrombosis; ocular complications. Neuropsychiatric manifestations. Pericarditis, myocarditis. | Hypopyon, retinal vasculitis, optic neuritis, eye vascular complication. Dural sinus thrombosis, aseptic meningitis and encephalitis. Arrhythmias. Superior and inferior vena cava syndrome. Abdominal aorta or pulmonary artery emergencies. Bowel perforation. |
| Churg–Strauss syndrome | Vasculitis with a multisystemic involvement. Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), allergic rhinitis, nasal obstruction, recurrent sinusitis, asthma, and peripheral blood eosinophilia. Systemic hypertension, pericarditis, abdominal pain, peripheral symmetric neuropathy; skin lesions and rash. | Heart failure, acute myocardial infarction, acute and constrictive pericarditis, GI bleeding, bowel perforation. |
| Dermatomyositis/polymyositis | Idiopathic inflammatory myopathies. Muscle weakness, myalgia, and muscle tenderness. Elevated serum creatine kinase. Systemic complaints (fever, malaise, ... |