Púrpura de Henoch-Schönlein: recorrência e cronicidade

Abstract

Objectives: To describe a group of patients treated at our service for Henoch-Schönlein purpura, with emphasis on recurrent and chronic cases, and to compare clinical and demographic characteristics of patients with monocyclic and recurrent disease. Methods:Data on 67 patients who had been treated since disease onset were analyzed. Twelve patients were excluded because they failed to return for follow-up consultations after less than 3 months, leaving a total of 55 children in the study sample. Recurrence was defined as the presence of a fresh episode after a period of at least 3 months without symptoms, and cases were defined as chronic when cutaneous, abdominal and renal manifestations persisted for a period of 12 months or more.Results: Recurrence was observed in 8/55 patients (14.4%) and four cases were chronic (7.2%). In 29/55 patients (52.7%), infection was identified as the trigger factor. A monocyclic clinical course was observed in 43 patients (26 of whom were girls, with a mean age of 5.4 years). Gastrointestinal and renal involvement was observed in 55.8 and 20.9% of patients, respectively. Among the 12 patients with recurrent or chronic Henoch-Schönlein purpura, three had arthritis, four exhibited signs and symptoms of abdominal involvement and seven of kidney disease: microscopic hematuria in five, macroscopic hematuria in one and hematuria with proteinuria in one other.Late onset was the only variable related to recurrence (p < 0.05). Conclusions:As is observed in medical literature, monocyclic cases are more common among children with early onset disease. Patients with Henoch-Schönlein purpura should be followed over the long term, since recurrent and chronic cases account for more than 20% of the total.

Henoch-Schönlein purpura with c-ANCA antibody in an adult

“…6 The lack of involvement of the respiratory tract and evidence of granulomatous infiltration - characteristic presentations of necrotizing vasculitides - reinforces the diagnosis of HSP with positive c-ANCA. 1,5,6 …”

“…3,4 Histopathology of skin demonstrates leukocytoclastic vasculitis associated with deposition of IgA on vessels. 5 As opposed to other vasculitides, such as granulomatous polyangiitis (Wegener's), microscopic polyangiitis and Churg-Strauss syndrome, positive markers for c-ANCA, class of cytoplasmic antineutrophil cytoplasmic antibodies, are rarely found in HSP. 6,7 …”

Pathophysiological Relationship between Infections and Systemic Vasculitis

“…Additionally, although the cause of IgA-mediated vasculitis is unknown, some infectious triggers have been identified. One study reported that 29 of 55 children [ 17 , 18 ] were isolated with Helicobacter pylori (49.3% of Chinese infants with IgA vasculitis compared to 23.4% of controls) [ 19 , 20 ]. Staphylococcus aureus [ 21 ] and Mycoplasma pneumoniae [ 22 ] are additional examples of these associations.…”

Childhood cutaneous vasculitis : A comprehensive appraisal

“…In a series of Brazilian children (n=55) with HSP, infection was identified as the trigger factor in 52.7% of the cases. [3] Some commonly used drugs in childhood may precipitate the occurrence of HSP; these include penicillin, erythromycin, sulphonamides, and anticonvulsants. It is an IgA mediated disorder and abnormality lies in the glycosylation of the hinge region of IgA1 that may be responsible for the pathogenesis.…”

“…[8] Gastrointestinal involvements were found to be more common (55.8%) than renal involvement (20.9%) in a recent study of children with HSP. [3] Very rarely, pulmonary hemorrhage may occur with a fatal outcome. [2]…”