Predictive factors for renal sequelae in adults with Henoch-Schönlein purpura.

OBJECTIVE: To examine the outcome and risk factors for renal sequelae in an unselected population of adults with Henoch-Schonlein Purpura (HSP). METHODS: Retrospective study of adult patients (> 20 years) with biopsy proved cutaneous vasculitis diagnosed as having HSP seen at a single center between 1984 and 1998. Patients were classified as having HSP according to proposed criteria. Only those patients with a followup of at least 1 year were included in this study of renal sequelae. RESULTS: Twenty-eight patients with a mean followup of 5.5 years fulfilled the inclusion criteria. When the study was concluded, 10 patients (36%) had renal sequelae and 2 (7%) had renal insufficiency. Men outnumbered women. However, neither a previous history of drugs, gender, nor age at disease onset was associated with a higher risk of permanent renal involvement. Patients with hematuria at disease onset or renal involvement during the course of the disease more commonly developed renal sequelae (p < 0.001). The presence of anemia (p = 0.05) at the time of diagnosis and the onset in summer (p < 0.05) were also more common in those with permanent renal involvement (renal sequelae). Patients with relapses had also a higher trend to develop renal sequelae (p = 0.07). All patients who fulfilled more than 2 of these 5 risk factors developed permanent renal involvement. With this model we were able to predict renal sequelae in 8 of the 10 patients who had this complication. The Goodman-Kruskal gamma test value was 0.92 (95% CI 0.78-1.00). CONCLUSION: In unselected adults with HSP, permanent renal involvement (renal sequelae) is not uncommon. Hematuria at disease onset and persistence of renal manifestations during the course of the disease are significant indicators of possible development of renal sequelae. These manifestations plus other features such as onset in summer, anemia at disease onset, or relapses of the disease may predict the development of renal sequelae in most patients.

Hypersensitivity vasculitis in adults: a benign disease usually limited to skin.

OBJECTIVES: To examine the clinical spectrum of hypersensitivity vasculitis (HV) in an unselected population of adults and establish differences between patients with HV limited to the skin and those with systemic involvement. METHODS: Retrospective study of adult patients (> 20 years) with biopsy-proven cutaneous leukocytoclastic vasculitis diagnosed as having HV, who were seen at the single hospital serving a well defined population between 1984 and 1998. Patients were classified as having HV according to the criteria of Michel et al. (9). To examine outcome and relapses of the disease only those patients with a follow-up of at least 1 year were included in this study. RESULTS: 64 patients with a mean follow-up of 4.9 +/- 3.5 (range: 1.1-13.6) years were studied. Ten (15.6%) had visceral involvement (3 gastrointestinal and 7 renal manifestations) during the course of the disease. The remaining patients had a leukocytoclastic vasculitis limited to the skin. When the study was concluded persistent hematuria and proteinuria was only observed in 1 patient and none developed renal insufficiency. Patients with a history of drug treatment and elevated ESR had more systemic complications but the difference was not statistically significant. The outcome was excellent in both patients with HV limited to the skin and in those with systemic complications during the course of the disease. CONCLUSIONS: In unselected adults HV is generally a benign disease confined exclusively to the skin. In those patients with systemic manifestations, visceral involvement is generally mild and transient.

Biopsy-negative giant cell arteritis: clinical spectrum and predictive factors for positive temporal artery biopsy.

OBJECTIVES: To examine the frequency and features of patients with biopsy-negative giant cell arteritis (GCA), establish differences with biopsy-proven GCA, and identify the optimal set of predictors for a positive temporal artery biopsy (TAB) in patients with GCA. METHODS: Retrospective study of an unselected population of patients with GCA diagnosed at the reference hospital for a defined population between 1981 and 1998. Patients were classified into biopsy-proven GCA if a TAB was positive for GCA, or biopsy-negative GCA if they fulfilled the American College of Rheumatology 1990 criteria for the classification of GCA (Hunder GG, et al Arthritis Rheum 1990; 33:1122-8) despite having a negative TAB. RESULTS: One hundred ninety Caucasian patients were diagnosed with GCA. Twenty-nine of them (15.3%) had a negative TAB. In these biopsy-negative patients, headache and polymyalgia rheumatica were frequent presenting symptoms. In contrast, jaw claudication, abnormal temporal artery on physical examination, and constitutional syndrome (asthenia, anorexia, and weight loss of 4 kg or more) were less common. They also had lower biologic markers of inflammation. The best predictive model of biopsy-proven GCA included a history of constitutional syndrome (OR = 6.1), an abnormal temporal artery on physical examination (OR = 3.2), and the presence of visual complications (OR = 4.9). CONCLUSIONS: In GCA, a subset of patients have a high likelihood of having a negative TAB. This subset seems to have less severe ischemic complications than that of biopsy-proven GCA. In patients without visual manifestations, abnormal temporal artery on examination or constitutional syndrome the risk of having an abnormal TAB is low.

Visual manifestations of giant cell arteritis. Trends and clinical spectrum in 161 patients.

Giant cell (temporal) arteritis (GCA) is the most common systemic vasculitis in Western countries. It involves large and medium-sized vessels with predisposition to the cranial arteries in the elderly. Cranial ischemic complications, in particular permanent visual loss, constitute the most feared aspects of this vasculitis. Although the use of corticosteroids and a higher physician awareness may have contributed to a decrease in the frequency of severe ischemic complications, permanent visual loss is still present in 7%-14% of patients. To investigate further the incidence, trends, and clinical spectrum of visual manifestations in patients with GCA, we examined the features of patients with biopsy-proven GCA diagnosed at the single reference hospital for a defined population in northwestern Spain during an 18-year period. Predictive factors for the development of any visual manifestation, not only permanent visual loss, were also examined. Between 1981 and 1998, 161 patients were diagnosed with biopsy-proven GCA. Visual ischemic complications were observed in 42 (26.1%), and irreversible blindness, mainly due to anterior ischemic optic neuropathy and frequently preceded by amaurosis fugax, was found in 24 (14.9%). Despite a progressive increase in the number of new cases diagnosed, there was not a significant change in the proportion of patients with visual manifestations during the study period (p = 0.37). Patients with visual ischemic complications had lower clinical and laboratory biologic markers of inflammation. Indeed, during the last years of the study, anemia was associated with a very low risk of visual complications. Also, HLA-DRB1*04-positive patients had visual manifestations more commonly. Patients with other ischemic complications developed irreversible blindness more frequently. The best predictors of any visual complication were HLA-DRB1*04 phenotype (odds ratio [OR] 7.47) and the absence of anemia at the time of admission (OR for patients with anemia = 0.07). The best predictors of irreversible blindness (permanent visual loss) were amaurosis fugax (OR 12.63) and cerebrovascular accidents (OR 26.51). The present study supports the claim that ocular ischemic complications are still frequent in biopsy-proven GCA patients from southern Europe. The presence of other ischemic complications constitutes an alarm for the development of irreversible blindness. In contrast, a higher inflammatory response may be a protective factor against the development of cranial ischemic events.