ABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinical manifestations, diagnosis and interventions of pulmonary hypertension (PAH) in patients with systemic lupus erythematosus (SLE).</p><p><b>METHODS</b>From January 2001 to December 2007, 798 SLE patients without prior diagnosis were admitted in our hospital, among whom 39 were identified to have concomitant PAH defined by echocardiography. The clinical data of the 39 cases were analyzed retrospectively.</p><p><b>RESULTS</b>The incidence of PAH was 4.9% in these cohort of SLE patients. The 39 SLE patients with concomitant PAH included 5 men and 34 women with a mean age of 34-/+12 years. Positive correlations were found between the occurrence of PAH and the Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia (P < 0.05). Patients with higher scores for SLE Disease Activity Index were liable to PAH. The presence of Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia is correlated to greater severity PAH with poor prognosis.</p><p><b>CONCLUSION</b>PAH is not a rare concomitant disease in SLE patients. The presence of Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia all suggest the likeliness of PAH in SLE patients, and echocardiographic examination may help derive an early diagnosis.</p>
Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , China , Epidemiology , Early Diagnosis , Echocardiography , Hypertension, Pulmonary , Diagnosis , Epidemiology , Lupus Erythematosus, Systemic , Diagnosis , Raynaud Disease , Retrospective StudiesABSTRACT
Objective Wegener's granulomatosis is a rare multisystem vasculitis of unknown aetiology. The clinical features of the disease,the treatment and long-term follow-up is observed in this study.Methods Eleven WG patients were from Rheumatology Department of Guangdong Province People's Hospital between 1999—2005.Data were obtained retrospectively.The patients' clinical manifestation and laboratory results were studied.Results All patients had upper and lower respiratory tracts involvement.The upper respiratory tract(nose,sinus,throat,trachea,and middle ear)-(E)lung(L),and kidneys(K)were the most commonly in- volved organs.The majority of patient received i.v.puls methylprednisolone 0.5 g/d?3 d,followed by oral pred- nisone 1 mg.kg~(-1).d~(-1)and i.v.pulses of cyclophosphamide 750 mg/m~2.The long-term outcome was good. Conclusion Wegner's granulomatosis is a systemic vasculitis occurring in patients with histopathologic mani- festation of necrotizing vasculitis with granuloma formation.Corticosteroid and immunosuppressive treatment may achieve good outcome.