Lupus-Associated Pulmonary Arterial Hypertension: Variable Course and Importance of Prompt Recognition

Table 2

Laboratory assessment of immunologic and inflammatory disease activity at admission and at follow-up 8 weeks later showing serologic phenotype and response to therapy.

Immunologic parameter (units, where applicable)	Reference range	Admission value	Postdischarge follow-up value
ESR (mm/hr)	0–20	35	46
CRP (mg/L)	0.1–3.0	27.9	9.2
C3 (mg/dL)	88–145	33	128
C4 (mg/dL)	16–39	<10	22
ANA titer	<1 : 40	>1 : 10,240	1 : 2,560
Anti-dsDNA Ab (IU/mL)	<12.5	25.7	<12.5
Anti-centromere Ab	<1 : 40	<1 : 40
Anti-SCL70	Negative	Negative
Anti-La Ab	Negative	Negative
Anti-Ro Ab	Negative	Positive
Anti-Smith Ab	Negative	Positive
Anti-RNP Ab	Negative	Positive
Anti-cardiolipin Ab (CU)	<20	10.6
Beta-2 glycoprotein (CU)	<20	14.9

Speckled pattern. Patients positive for anti-dsDNA and anti-Smith had better response to immunosuppressive therapy during an SLE-associated PAH flare [10]. Anti-RNP and anti-cardiolipin positivity correlates with evidence of PH on echocardiogram [11]. Ab: antibody; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; ANA: antinuclear antibody; dsDNA: double-stranded DNA; RNP: ribonucleoprotein.