| Anti-phospholipid syndrome | Multiple and recurrent venous and arterial thromboses, recurrent abortions. Secondary form is associated with SLE, RA, systemic sclerosis, and Sjögren syndrome. Thrombophlebitis and DVT, pulmonary embolism, thrombocytopenia, hemolytic anemia, livedo reticularis, stroke, transient ischemic attack, eye vascular complications. Coronary, renal, mesenteric and stroke, ARDS. |
| Ankylosing spondylitis | Chronic inflammatory disease of the axial skeleton, with progressive stiffness of the spine. Young adults (peak at 20 and 30 years). Back pain (improves with exercise), buttock, hip, or shoulder pain, systemic complaints (fever, malaise, fatigue, weight loss, myalgias), uveitis, restrictive pulmonary failure due to costovertebral rigidity, renal impairment, fracture of the ankylosed spine, acute spinal cord compression. |
| Adult still disease | Inflammatory disorder. Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), arthritis, myalgia, evanescent rash, pharyngitis, lymphadenopathy, splenomegaly, anemia, thrombocytopenia. Pericarditis, myocarditis, pleurisy, ARDS, arrhythmias, heart and liver failure. |
| Behçet disease | Chronic, relapsing, inflammatory disease. Systemic vasculitis involving arteries and veins of all sizes (carotid, pulmonary, aortic, and inferior extremities 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 DVT; ocular complications. Neuropsychiatric manifestations. Pericarditis, myocarditis, bowel perforation. |
| Churg-Strauss syndrome | Vasculitis with a multisystemic involvement. Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), allergic rhinitis, recurrent sinusitis, asthma, and peripheral blood eosinophilia. Systemic hypertension, pericarditis, abdominal pain, peripheral neuropathy; skin lesions, AMI, bowel perforation. |
| Dermatomyositis/polymyositis | Idiopathic inflammatory myopathies. Muscle weakness, myalgia and muscle tenderness. Elevated serum CK. Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), Raynaud phenomenon, nonerosive inflammatory polyarthritis, esophageal dysfunction, respiratory failure, aspiration lung infections, conduction disturbances. |
| Giant cell arteritis (temporal arteritis) | Chronic vasculitis of large and medium sized vessels. Elderly (mean age at diagnosis: 70 years). 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. |
| Henoch-Schönlein purpura | Systemic vasculitis associated with IgA deposition, generally in children. Palpable purpura (in patients with neither thrombocytopenia nor coagulopathy), arthritis/arthralgia, abdominal pain, GI bleeding, and renal impairment (adult). |
| 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. |
| 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, leucocytosis and anemia, stroke, mesenteric ischemia, acute scrotum. |
| Rheumatoid arthritis | Chronic, systemic, inflammatory disorder. Symmetrical and potentially destructive arthritis. Systemic symptoms (fever, malaise, fatigue, weight loss, myalgia), skin lesions, splenomegaly. Cervical spine involvement, pleuritis, pericarditis, myocarditis, and aortitis. Cricoarytenoid arthritis with potential for airway obstruction. Ocular involvement. Peripheral artery disease, Sjögren syndrome, vasculitis, and renal impairment. Abdominal pain. Anemia, leucopenia, thrombocytosis, and Felty syndrome. ACS, respiratory failure. |
| Polymyalgia rheumatica | Immune-mediated condition. Ears (violaceous and erythematous auricula), nose (saddle nose deformity), and other cartilaginous structures inflammation (especially eyes, joints, and respiratory tract). One-third of cases associated with another SRD. Sternoclavicular, costochondral, and manubriosternal arthritis, upper airway involvement, aortic or mitral valvular regurgitation, pericarditis, renal impairment, peripheral neuropathies, ocular complications. |
| 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, the wrists and the knees), butterfly rash, mucocutaneous manifestations, oral and/or nasal ulcers, Raynaud phenomenon. Neuropsychiatric manifestations, pleurisy, lupus pneumonitis, shrinking or vanishing lung syndrome, and pulmonary hypertension. Libman-Sacks endocarditis, pericarditis, myocarditis, endocarditis. GI unspecific complaints. Renal impairment, leucopenia, mild anemia, and thrombocytopenia. Ocular complications, ACS. |
| Sjögren syndrome | Autoimmune disease. May be primary; secondary form is mostly associated with RA, SLE, polymyositis, or dermatomyositis. Systemic symptoms, arthralgia, skin lesions, Raynaud phenomenon. Pulmonary hypertension, pericarditis, neuropsychiatric manifestations, peripheral neuropathy, hepatic abnormalities, renal impairment, hypokalemic respiratory arrest, stroke, pulmonary embolism, transverse myelitis. |
| Systemic sclerosis (scleroderma) | Inappropriate and excessive accumulation of collagen in a variety of tissues; widespread vascular lesions with vascular spasm, thickening of the vascular wall and narrowing of the lumen. Systemic complaints (fever, malaise, fatigue, weight loss, myalgia), skin lesions (fingers, hands and face), carpal tunnel syndrome, Raynaud phenomenon. Renal impairment, GI dysmotility, gastroesophageal reflux (aspiration pneumonitis), chronic esophagitis and stricture formation. Vascular ectasia in the stomach (“watermelon stomach”). Alveolar hemorrhage, ARDS, arrhythmias, scleroderma renal crisis. |
| Takayasu 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. Normochromic, normocytic anemia, ACS, bowel ischemia and perforation, stroke. |
| Wegener granulomatosis | Multiple organ system vasculitis and necrotizing granulomas. Respiratory tract manifestations in approximately 100% cases, with nose, oral cavity, upper trachea, external and middle ear, and orbits, 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). Pericarditis, myocarditis. Renal impairment. Anemia, leukocytosis and thrombocytosis, ACS. |