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Differential Diagnosis
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The exclusion of other autoimmune disorders, particularly those in the SLE spectrum, is essential, as such disorders may be associated with additional complications requiring alternative treatments Other genetic or acquired conditions associated with hypercoagulability such as protein C, protein S, factor V Leiden, and antithrombin III deficiency should be excluded The thrombocytopenia associated with APS must be distinguished from immune thrombocytopenic purpura Catastrophic APS has a broad differential, including sepsis, pulmonary-renal syndromes, systemic vasculitis, disseminated intravascular coagulation, and thrombotic thrombocytopenic purpura
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Treatment
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Present recommendations for anticoagulation are to treat patients with warfarin to maintain an INR of 20 30 Patients who have recurrent thrombotic events on this level of anticoagulation may require higher INRs (> 30), but the bleeding risk increases substantially with this degree of anticoagulation Guidelines indicate that patients with APS should be treated with anticoagulation for life Because of the teratogenic effects of warfarin, subcutaneous heparin and baby aspirin is the usual approach to prevent pregnancy complications in women with APS In patients with catastrophic APS, a three-pronged approach is taken in the acute setting: intravenous heparin, high doses of corticosteroids, and either intravenous immune globulin or plasmapheresis
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Bertolaccini ML et al Antiphospholipid antibody testing: which are most useful for diagnosis Rheum Dis Clin North Am 2006 Aug;32(3):455 63 [PMID: 16880078] Finazzi G et al A randomized clinical trial of high-intensity warfarin vs conventional antithrombotic therapy for the prevention of recurrent thrombosis in patients with the antiphospholipid syndrome (WAPS) J Thromb Haemost 2005 May;3 (5):848 53 [PMID: 15869575]
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In early attacks of RP, only one or two fingertips may be affected; as it progresses, all fingers down to the distal palm may be involved The thumbs are rarely affected During recovery there may be intense rubor, throbbing, paresthesia, pain, and slight swelling Attacks usually terminate spontaneously or upon returning to a warm room or putting the extremity in warm water The patient is usually asymptomatic between attacks Sensory changes that often accompany vasomotor manifestations include numbness, stiffness, diminished sensation, and aching pain Primary RP appears first between ages 15 and 30, almost always in women It tends to be mildly progressive and, unlike secondary RP (which may be unilateral and may involve only one or two fingers), symmetric involvement of the fingers of both hands is the rule Spasm becomes more frequent and prolonged Unlike secondary RP, primary RP does not cause digital pitting, ulceration, or gangrene Nailfold capillary abnormalities are among the earliest clues that a person has secondary rather than primary RP The nailfold capillary pattern can be visualized by placing a drop of grade B immersion oil at the patient s cuticle and then viewing the area with an ophthalmoscope set to 20 40 diopters Dilation or dropout of the capillary loops indicates the patient has a secondary form of RP, most commonly scleroderma (Table 20 12) While highly specific for secondary RP, nailfold capillary changes have a low sensitivity Digital pitting or ulceration or other abnormal physical findings (eg, skin tightening, loss of extremity pulse, rash, swollen joints) can also provide evidence of secondary RP
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ESSENTIALS OF DIAGNOSIS
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Paroxysmal bilateral symmetric pallor and cyanosis followed by rubor of the skin of the digits Precipitated by cold or emotional stress; relieved by warmth Primarily affects young women Primary form benign; secondary form can cause digital ulceration or gangrene
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Differential Diagnosis
Primary RP must be differentiated from the numerous causes of secondary RP (Table 20 12) The history and examination may suggest the diagnosis of systemic sclerosis (including its CREST variant), SLE, and mixed connective tissue disease; RP is occasionally the first manifestation of these disorders The diagnosis of many of these rheumatic diseases can be confirmed with specific serologic tests (see Table 20 10) The differentiation from Buerger disease (thromboangiitis obliterans) is usually not difficult, since thromboangiitis
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