Differentiating Antiphospholipid syndrome from other diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Feham Tariq, MD [2]

Overview

Antiphospholipid syndrome should be differentiated from conditions such as hemolytic uremic syndrome-thrombotic thrombocytopenic purpura (HUS-TTP), disseminated intravascular coagulation (DIC), and vasculitis .

Differentiating Antiphospholipid antibody syndrome from other Diseases

• Hemolytic uremic syndrome-Thrombotic thrombocytopenic purpura (HUS-TTP)
• Disseminated Intravascular Coagulation (DIC)
• Vasculitis
• Embolic disease
• Cryoglobulinemia
• Calciphylaxis

Abbreviations: ABG= Arterial blood gas, ANA= Antinuclear antibody, ANP= Atrial natriuretic peptide, ASO= Antistreptolysin O antibody, BNP= Brain natriuretic peptide, CBC= Complete blood count, COPD= Chronic obstructive pulmonary disease, CRP= C-reactive protein, CT= Computed tomography, CXR= Chest X-ray, DVT= Deep vein thrombosis, ESR= Erythrocyte sedimentation rate, HRCT= High Resolution CT, IgE= Immunoglobulin E, LDH= Lactate dehydrogenase, PCWP= Pulmonary capillary wedge pressure, PCR= Polymerase chain reaction, PFT= Pulmonary function test.

Diseases			Clinical manifestations							Para-clinical findings							Gold standard	Additional findings
			Symptoms				Physical examination
										Lab Findings				Imaging		Histopathology
			Dyspnea	Fever	Weight loss	Arthralgia	Skin manifestation	Organomegaly	Focal neurological disorder	CBC	ESR	Antibodies	Other	CT scan	Other
Antiphospholipid syndrome			+	+	-	-	Livedo reticularis, palpable purpura, leg ulcers, raynaud phenomena	hepatomegaly, splenomegaly	Stroke, transient ischemic attack	Schistocytes on peripheral smear,Thrombocytopenia, Hemolytic anemia	↑	Lupus anticoagulant,anticardiolipin antibodies	-	Stroke, Pulmonary embolism, Budd-Chiari syndrome	-	Noninflammatory bland thrombosis without perivascular inflammation	Hx of thrombosis and antiphospholipid antibodies	Miscarriage, Pulmonary hypertension
Systemic lupus erythematosus (SLE)
Thrombosis	Thrombophilia	DIC
		HUS	+	+	-	+		Liver/spleen swelling	+	Anemia, Thrombocytopenia, Reticulocytosis	↑	ADAMTS13	Increased lactate dehydrogenase	Thalami, brainstem, or cerebellumabnormality		Microthrombosesinclude fibrin thrombi that may occlude the glomerular tufts	Clinical findings coupled with laboratory abnormalities	Hematuria, Proteinuria
		ITP
		HELLP
	Paroxysmal nocturnal hemoglobinuria (PNH)
	Heparin-induced thrombocytopenia (HIT)
	Myeloproliferative diseases
Recurrent pregnancy loss	Uterine abnormality
	Chromosomal abnormality
	Hypothyroidism