Characterization of scrotal involvement in children and adolescents with IgA vasculitis.

OBJECTIVE: To characterize scrotal involvement in children and adolescents with IgA vasculitis. METHODS: A cross-sectional retrospective study included 296 IgA vasculitis (EULAR/PRINTO/PRES criteria) patients, 150/296 (51%) were males and assessed by demographic/clinical/laboratory and treatments. Scrotal involvement was defined by the presence of scrotal edema and/or pain/tenderness in physical examination and/or testicular Doppler ultrasound abnormalities. RESULTS: Scrotal involvement was observed in 28/150 (19%) IgA vasculitis patients. This complication was evidenced at IgA vasculitis diagnosis in 27/28 (96%). Acute recurrent scrotal involvement was observed in 2/150 (1%) and none had chronic subtype. Further analysis of patients with scrotal involvement at first episode (n = 27) compared to those without this complication (n = 122) revealed that the median age at diagnosis [4.0 (2.0-12) vs. 6 (1.3-13) years, p = 0.249] was similar in both groups. The frequency of elevated serum IgA was significantly lower in IgA vasculitis patients with scrotal involvement versus without this manifestation (18% vs. 57%, p = 0.017), whereas glucocorticoid (93% vs. 49%, p < 0.0001) and ranitidine use (63% vs. 30%, p = 0.003) were significantly higher in the former group. CONCLUSIONS: The scrotal involvement occurred in almost one fifth of IgA vasculitis patients and was commonly evidenced as acute subtype at diagnosis. Scrotal signs/symptoms improved after a prompt use of glucocorticoid and was associated with low frequency of elevated IgA serum levels.

Features of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study.

OBJECTIVE: To evaluate demographic data and clinical and laboratory features at disease diagnosis in 3 different age groups of childhood-onset systemic lupus erythematosus (SLE): group A, early-onset (<6 years); group B, school age (≥6 to <12 years); and group C, adolescent (≥12 to <18 years). METHODS: This was a Brazilian multicenter cohort retrospective study in 10 pediatric rheumatology centers, including 847 childhood-onset SLE patients. RESULTS: Patients were divided into 3 groups: group A with 39 patients (4%), group B with 395 patients (47%), and group C with 413 patients (49%). Of 39 childhood-onset SLE patients in group A, 3 (8%) were ages <2 years, 4 (10%) were ≥2 to <3 years, and 32 (82%) were ≥3 and <6 years. A total of 74 childhood-onset SLE patients were analyzed for C1q levels, and complete C1q deficiency was observed in 3 of 74 patients (4%), all in group A. Groups were similar regarding high frequencies of female sex, nephritis, neuropsychiatric involvement, Systemic Lupus Erythematosus Disease Activity Index 2000 score ≥8, autoantibody profile, elevated acute phase proteins, and low complement levels (P > 0.05). However, the frequency of fever (78% versus 61% versus 47%; P < 0.0001), hepatomegaly (42% versus 29% versus 14%; P < 0.0001), splenomegaly (28% versus 12% versus 4%; P < 0.0001), and discoid lupus (13% versus 4% versus 4%; P = 0.020) was significantly higher in group A compared to groups B and C. The frequency of weight loss >2 kg (19% versus 28% versus 36%; P = 0.017), photosensitivity (34% versus 41% versus 51%; P = 0.006), leukopenia <4,000/mm3 (14% versus 25% versus 30%; P = 0.048), and lymphopenia <1,500/mm3 (22% versus 41% versus 47%; P = 0.011) was significantly lower in group A. CONCLUSION: Our large multicenter study identified the finding that the initial appearance of childhood-onset SLE is characterized by comparable high frequency of internal organ involvement and some distinct clinical and laboratory features in early-onset and adolescent groups.

Severe intestinal involvement as initial manifestation of systemic childhood polyarteritis nodosa: report of two cases.

Systemic childhood polyarteritis nodosa (C-PAN) is a rare primary vasculitis involving medium or small sized arteries. Abdominal angina is an important and serious complication of PAN, occurring usually 15 to 30 min after food intake, and particularly in adult patients. However, to our knowledge, this involvement as the first manifestation of C-PAN was not described. Therefore, we reported herein two C-PAN cases that fulfilled the new criteria for this vasculitis. These patients were young boys that had malignant arterial hypertension and recurrent post-prandial cramping with acute abdomen. Both of them were submitted to laparotomy that revealed multiple and diffuse intestinal necrosis. One of our cases had a severe post-prandial cramping, even after drinking water, and the laparotomy evidenced multiple intestinal perforations. In spite of use of antihypertensive therapies, immunosuppressive agents (corticosteroids, cyclophosphamide and/or methotrexate) and intravenous immunoglobulin, they died possibly due to severe and disseminated activity disease. In conclusion, we described herein the first two fatal cases of C-PAN that presented severe abdominal pain as initial manifestation. We suggest that the diagnosis of PAN should be considered in patients under acute abdominal angina with no apparent etiology.