ABSTRACT
Lupus enteritis (LE) is a rare presentation of systemic lupus erythematosus manifesting with nonspecific symptoms, laboratory derangements, and imaging findings. Rarely, LE may progress to bowel perforation, hemorrhage, and even death. Treatment with systemic glucocorticoids often results in rapid clinical improvement, but patients may require further immunosuppression. We present a case of severe LE complicated by life-threatening intractable gastrointestinal hemorrhage and warm autoimmune hemolytic anemia refractory to glucocorticoids and ultimately requiring massive transfusions, intravenous immunoglobulin, and plasmapheresis. This case illustrates the importance of early specialist involvement and treatment with intravenous immunoglobulin and plasmapheresis for severe, life-threatening LE.
ABSTRACT
Hydralazine, an arterial vasodilator, is a widely used medication for the management of hypertension and heart failure, especially for patients who cannot tolerate the use of ACEIs or ARBs. It is generally well-tolerated and has a safe profile in pregnancy. However, hydralazine can induce immune-mediated side effects, such as hydralazine-induced lupus and less commonly hydralazine- induced ANCA vasculitis. The latter most commonly affects the kidneys with or without other organ involvement. There are several cases reported in the literature of hydralazine-induced ANCA associated vasculitis (AAV) that have pulmonary manifestations, also known as hydralazine- induced pulmonary-renal syndrome (PRS), a condition with a high risk of mortality. We are reporting a case of Hydralazine-induced ANCA associated glomerulonephritis with severe diffuse alveolar hemorrhage (DAH). In addition, we will review the current literature and discuss the importance of prompt diagnosis and early management to decrease mortality and morbidity associated with this serious condition.
Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Glomerulonephritis , Hemorrhage , Hydralazine/adverse effects , Lung Diseases , Vasodilator Agents/adverse effects , Aged , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/chemically induced , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/pathology , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/therapy , Female , Glomerulonephritis/chemically induced , Glomerulonephritis/diagnosis , Glomerulonephritis/pathology , Glomerulonephritis/therapy , Heart Failure , Hemorrhage/chemically induced , Hemorrhage/diagnosis , Hemorrhage/pathology , Hemorrhage/therapy , Humans , Hydralazine/therapeutic use , Hypertension/drug therapy , Lung/diagnostic imaging , Lung/pathology , Lung Diseases/chemically induced , Lung Diseases/diagnosis , Lung Diseases/pathology , Lung Diseases/therapy , Vasodilator Agents/therapeutic useABSTRACT
BACKGROUND/PURPOSE: First-degree relatives (FDRs) of patients with ankylosing spondylitis (AS) may be at high risk of spondyloarthritis. We examined the frequency, characteristics of chronic back pain (CBP), associated features, persistence of symptoms, and HLA-B27 allele frequency in FDRs of AS patients, also comparing those FDRs with participants in NHANES 2009-2010 with CBP. METHODS: 399 FDRs of AS probands were divided into: (1) No CBP (subjects >40 years old at study visit without CBP) (n=162); (2) NICBP (non-inflammatory CBP) (n=82), and (3) CIBP (inflammatory CBP) (n=155). White FDRs with CBP were compared with 772 participants in NHANES 2009-2010 with CBP. FDRs were invited to return for reassessment. RESULTS: FDRs with CIBP had earlier onset of CBP than those with NICBP (p<0.001) and had higher frequency of heel pain than those without CBP (p=0.002). HLA-B27 occurred in 57% of FDRs with CIBP vs 39.6% of those without CBP (p=0.005, OR=1.9). Of 23 patients with CIBP at baseline re-evaluated 67.04±31.02 months later, 16 (73%) still had CIBP, whereas 4 (31%) of 13 NICBP patients seen 61.23±31.84 months later remained symptomatic. CONCLUSION: CIBP in FDRs of AS patients is HLA-B27-associated, has earlier onset and tends to persist compared to NICBP.
Subject(s)
Back Pain , HLA-B27 Antigen , Spondylarthritis , Spondylitis, Ankylosing , Adult , Back Pain/complications , Humans , Male , Nutrition Surveys , Spondylitis, Ankylosing/complicationsABSTRACT
BACKGROUND: Systemic Mastocytosis (SM) is a disorder of excessive mast cell infiltration in multiple organ tissues. Atherosclerosis is a major risk factor for developing acute coronary syndrome. In addition to lipid accumulation in the arterial wall, inflammation plays an important role in the pathogenesis of plaque rupture and activating the thrombosis cascade. The Mast cells contribution to plaque destabilization has been well established in multiple animal and human studies. In a recent study, SM has been proven to be associated with a higher incidence of acute coronary syndrome even with lower plasma lipids levels. The study showed that 20% of patients with SM had cardiovascular events compared to only 6% in the control group with adjustment to all cardiac risk factors. CASE: We presented a patient with no risk factors for heart disease other than old age and history of SM who developed acute myocardial infarction. CONCLUSION: SM can be life-threatening and can result in ACS, anaphylactic reaction, syncope, or cardiac arrest. Clinicians should have a high index of suspicion of acute coronary syndrome (ACS) occurrence in the setting of inflammatory conditions, such as SM and KS, and vice versa, where SM should be considered or ruled out in patients who suffer from anaphylaxis and cardiac arrest or myocardial infarction.