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), 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, fatigue, weight loss, myalgia), Raynaud phenomenon, nonerosive inflammatory polyarthritis, esophageal dysfunction, ILD, aspiration lung infections. | ARDS. Respiratory failure and arrest due to diaphragmatic or chest wall muscle weakness, alveolar hemorrhage, and ILD. Heart failure, arrhythmias, and conduction disturbances. |
Giant cell arteritis (temporal arteritis) | Chronic vasculitis of large- and medium-sized vessels. Elderly (mean age at diagnosis: 70 years old). Associated with polymyalgia rheumatica in 50% of cases. Localized headache of new onset, tenderness of the temporal artery, and biopsy revealing a necrotizing arteritis. Temporal artery may be normal on clinical examination. Gradual onset, systemic complaints, jaw or tongue claudication, eye complaints and visual loss. Aortic regurgitation and aortic arch syndrome. Neurologic complications due to carotid and vertebrobasilar vasculitis. | Ischemic optic neuropathy, eye vessel occlusion. Aortitis (especially the thoracic tract) and aortic emergencies. Stroke. |
Henoch-Schönlein purpura | Systemic vasculitis associated with immunoglobulin A deposition, generally in children. Frequently, acute presentation follows an upper respiratory infection. Palpable purpura (in patients with neither thrombocytopenia nor coagulopathy), arthritis/arthralgia, abdominal pain, and renal impairment (adult), ILD. | Respiratory failure and alveolar hemorrhage. Seizures, intracranial bleeding, GI hemorrhage, bowel ischemia or perforation, acute pancreatitis, intussusception (children). Acute scrotum. |
Microscopic polyangiitis | Small-vessel systemic vasculitis, characterized by rapidly progressive glomerulonephritis and pulmonary involvement. Lung complications differentiate microscopic polyangiitis from polyarteritis nodosa. Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), arthralgias, skin lesions, hemoptysis, abdominal pain, renal impairment, systemic hypertension. | Rapidly progressive glomerulonephritis, severe lung hemorrhage, GI bleeding. |
Polyarteritis nodosa | Systemic necrotizing vasculitis of the medium-sized muscular arteries. Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), arthralgias, skin lesions, abdominal pain, renal impairment, systemic hypertension, peripheral mononeuropathy typically with both motor and sensory deficits, eye complications, leukocytosis, and normochromic anemia. | Acute scrotum, ischemic and hemorrhagic stroke, acute coronary syndrome, heart failure, peripheral artery ischemia, mesenteric ischemia and bowel perforation, GI bleeding, acute pancreatitis, malignant hypertension. |
Relapsing polychondritis | Immune-mediated condition. Ears (violaceous and erythematous auricula), nose (saddle nose deformity), and other cartilaginous structures inflammation (especially joints and respiratory tract). One-third of cases associated with other systemic rheumatic diseases. Sternoclavicular, costochondral, and manubriosternal arthritis, upper airway involvement, aortic or mitral valvular regurgitation, pericarditis, renal impairment, peripheral neuropathies, ocular complications. | Airway obstruction. Acute renal failure, aortitis and aortic emergencies, heart block, ACS, scleritis, peripheral ulcerative keratitis, and acute scrotum. |
Rheumatoid arthritis | Chronic, systemic, inflammatory disorder. Symmetric and potentially destructive arthritis. Systemic symptoms (fever, malaise, fatigue, weight loss, myalgia), skin lesions, splenomegaly. Cervical spine involvement, pleuritis, ILD, pericarditis, myocarditis, and aortitis. Cricoarytenoid arthritis with potential for airway obstruction, ocular involvement. Peripheral artery disease, Sjögren's syndrome, vasculitis, and renal impairment. Abdominal pain. Anemia, leukopenia, thrombocytosis, and Felty's syndrome. Increased risk of lymphoproliferative diseases, particularly non-Hodgkin's lymphoma. | Airway obstruction, obliterative bronchiolitis, acute respiratory failure. ACS, heart failure, thoracic aorta dissection, arrhythmias and conduction disturbances, subluxation of the atlantoaxial joints, bowel ischemia and perforation. Septic arthritis. Scleritis. |
Systemic lupus erythematosus | Systemic autoimmune disease, characterized by relapses and remissions, and affecting virtually every organ. Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), symmetric and polyarticular arthritis (small joints of the hands, wrists, and knees), butterfly rash, mucocutaneous manifestations, oral and/or nasal ulcers, Raynaud's phenomenon. Neuropsychiatric manifestations, pleurisy, lupus pneumonitis, shrinking or vanishing lung syndrome, ILD, and pulmonary hypertension. Libman-Sacks endocarditis, pericarditis, myocarditis, endocarditis. GI unspecific complaints. Renal impairment, leukopenia, mild anemia, and thrombocytopenia. Antiphospholipid syndrome. Ocular complications. | Airway obstruction, ARDS, respiratory failure and arrest, alveolar hemorrhage, ACS, cardiac tamponade, heart failure, arrhythmias, pulmonary embolism, stroke, acute renal failure, Guillain-Barré–like syndrome, transverse myelitis, seizures. Bowel ischemia and perforation, GI bleeding, acute pancreatitis. Hemolytic anemia, thrombotic microangiopathic hemolytic anemia. |
Sjögren's syndrome | Autoimmune disease. May be primary; secondary form is mostly associated with rheumatoid arthritis, systemic lupus erythematosus, polymyositis, or dermatomyositis. Xerophthalmia and xerostomia, systemic symptoms, arthralgia, skin lesions, Raynaud's phenomenon. ILD, pulmonary hypertension, pericarditis, neuropsychiatric manifestations, peripheral neuropathy, hepatic abnormalities, renal impairment, increased risk of non-Hodgkin's lymphoma. | Hypokalemic respiratory arrest. Heart block, pulmonary embolism, ischemic stroke, transverse myelitis, optic neuritis, renal tubular acidosis, acute pancreatitis. |
Systemic sclerosis (scleroderma) | Inappropriate and excessive accumulation of collagen and matrix in a variety of tissue; widespread vascular lesions with endothelial dysfunction, vascular spasm, thickening of the vascular wall and narrowing of the vascular lumen. Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), skin lesions (fingers, hands, and face), carpal tunnel syndrome, Raynaud's phenomenon. ILD, renal impairment, GI dysmotility, gastroesophageal reflux (aspiration pneumonitis), chronic esophagitis and stricture formation. Vascular ectasia in the stomach ("watermelon stomach"). | Scleroderma renal crisis. Respiratory failure, ARDS, aspiration pneumonitis, pulmonary hypertension, alveolar hemorrhage, heart failure, arrhythmias, and conduction disturbances. |
Takayasu's arteritis | Chronic vasculitis, young women, predominantly Asians. Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), arthralgias, skin lesions, abdominal pain and diarrhea. Aorta and its primary branches, and pulmonary artery involvement. Neurologic manifestations, syncope, subclavian steal syndrome, extremities ischemia. Renovascular hypertension. Normochromic normocytic anemia. | ACS, bowel ischemia and perforation, GI bleeding, stroke. |
Wegener's (granulomatosis with polyangiitis) | Multiple organ system vasculitis and necrotizing granulomas. Respiratory tract manifestations in approximately of 100% cases, with nose, oral cavity, upper trachea, external and middle ear, and orbit inflammations. Upper airway and pulmonary manifestations. Constitutional symptoms, arthralgias, glomerulonephritis and small vessel vasculitis (scleritis and episcleritis, palpable purpura or cutaneous nodules, peripheral neuropathy, deafness). Systemic hypertension. Pericarditis, myocarditis. Renal impairment. Anemia, leukocytosis, and thrombocytosis. | Airway obstruction, subglottic stenosis, bronchiolitis obliterans organizing pneumonia, and alveolar hemorrhage. ACS, arrhythmias. Rapidly progressive glomerulonephritis. |