ABSTRACT
OBJECTIVE: To document changes in pulmonary arterial systolic pressure (PASP) in patients with SLE who have received CYC for any indication. METHODS: Twenty-four patients with SLE pulmonary arterial hypertension (PAH) with a PASP of >30 mmHg by transthoracic echocardiography received i.v. CYC (n = 24) or steroids (n = 24) with or without vasodilators (n = 20). Baseline clinical characteristics and PASP were evaluated before and after therapy at 6 months. Responders were defined as those who had a decrease in PASP of >15 mmHg from baseline along with improvement in their New York Heart Association functional class. RESULTS: There were 11 responders (45.83%), with a decrease in mean PASP from 59.33 mmHg at baseline to 43.29 mmHg at the end of 6 months (P < 0.0001). The decrease in mean PASP from 39.75 mmHg at baseline to 34.4 mmHg at the end of 6 months was significant in four patients who received immunosuppression alone (P = 0.04). There was no difference in baseline PASP and disease activity between responders and non-responders. Two deaths were noted. CONCLUSION: Immunosuppression and vasodilators produced significant improvement in SLE PAH over 6 months.
Subject(s)
Hypertension, Pulmonary/drug therapy , Hypertension, Pulmonary/etiology , Immunosuppressive Agents/therapeutic use , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Vasodilator Agents/therapeutic use , Adolescent , Adult , Blood Pressure/drug effects , Blood Pressure/physiology , Cohort Studies , Cyclophosphamide/pharmacology , Cyclophosphamide/therapeutic use , Drug Therapy, Combination , Echocardiography , Female , Humans , Hypertension, Pulmonary/physiopathology , Immunosuppressive Agents/pharmacology , Lupus Erythematosus, Systemic/physiopathology , Steroids/pharmacology , Steroids/therapeutic use , Systole/drug effects , Systole/physiology , Treatment Outcome , Vasodilator Agents/pharmacology , Young AdultABSTRACT
One of the rare causes of secondary vasculitides is malignancy. Hematological malignancies produce secondary vasculitis more frequently than solid malignancies. Here in we report a case of acute myeloid leukemia presenting with anti-neutrophil cytoplasmic antibody-positive vasculitis. This case highlights the importance of looking for underlying malignancies, especially leukemias in patients presenting with features of systemic vasculitides.
Subject(s)
Leukemia, Myeloid, Acute/diagnosis , Paraneoplastic Syndromes/diagnosis , Vasculitis, Leukocytoclastic, Cutaneous/diagnosis , Antibodies, Antineutrophil Cytoplasmic/blood , Diagnosis, Differential , Female , Glucocorticoids/therapeutic use , Humans , Leukemia, Myeloid, Acute/blood , Leukemia, Myeloid, Acute/complications , Paraneoplastic Syndromes/blood , Paraneoplastic Syndromes/complications , Prednisolone/therapeutic use , Vasculitis, Leukocytoclastic, Cutaneous/blood , Vasculitis, Leukocytoclastic, Cutaneous/complications , Vasculitis, Leukocytoclastic, Cutaneous/drug therapyABSTRACT
Behcet's syndrome is an inflammatory disorder of unknown cause, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Behcet's syndrome with predominant vascular involvement is known as vasculo-Behcet. Arterial complications occur in only 1 to 7% of patients with Behcet's syndrome. In most reports, arterial lesions are isolated. We report a case of Behcet's syndrome that, over 6 years, developed multiple aneurysms in peripheral arteries and aorta without any coexisting venous thrombosis. An increased awareness of Behcet's syndrome and its vascular complications is essential. This is highlighted by the fact that our patient had to undergo four surgeries and many years of diagnostic uncertainty before reaching at the final diagnosis.
