Case 2 investigation was initially prompted by nonspecific musculoskeletal symptoms, despite that additional findings were quickly found out. 2 years. Despite hypogammaglobulinemia (minimum of 496?mg/dL), no infections or complications were reported, and immunoglobulin alternative therapy was not required. During the 43 weeks of follow-up, there is a reference to slight sinusitis symptoms with no further issues. Pulmonary function checks and renal function remained normal. Regular analytical monitoring shown normalization of ESR and a progressive decrease of C-ANCA titers. Open in a separate window Number 1 CT scan showing parenchymal nodular areas with internal cavitation (a, b) with almost complete resolution after 4 weeks (c, d), Case 1. Case TEMPOL 2 . A 12-year-old Caucasian woman with a history of asthma, with no familial history of known rheumatic diseases, was admitted in the emergency division with inflammatory indications of the posterior section of the thigh and right wrist lasting the previous 10 days and sporadic issues of lower limb myalgia enduring several months. Fever, having started that day, was also present. At physical exam, she was apyrexial, hemodynamically stable, normotensive, but pale. She weighed 35?kg, and her height was 146?cm. Cardiac evaluation exposed a systolic murmur II/VI (aortic focus), and inflammatory indications within the infragluteal region of the right thigh (10?cm of size) and on the right wrist (2-3?cm in diameter) were present. Ultrasound suggested myositis of the thigh and tenosynovitis of the wrist, and laboratory screening shown a normocytic normochromic anemia (Hg 9.4?g/dL), mild leukocytosis (14360/prophylaxis with cotrimoxazole was started. There was a progressive control of pulmonary hemorrhage and hematoproteinuria, with maintained renal function. Despite reference to self-limited episodes of epistaxis, ENT evaluation was normal. Ophthalmologic evaluation did not CTMP show any indications of uveitis or additional lesions. During outpatient follow-up, she completed 4 weeks of rituximab having a progressive tapering of glucocorticoids to 5?mg/day time (prednisolone) and cycles of rituximab every 6 months, for 18 months. Despite the absence of reported infections, she managed a prolonged hypogammaglobulinemia (minimum amount 469?mg/dL), and immunoglobulin alternative therapy was administered. Pulmonary function checks showed a slight obstructive pattern that improved over time. Clinical remission was accomplished 1 month after the onset of the disease, and no additional significant complications were reported during the 43 weeks of follow-up. 3. Conversation GPA is definitely a systemic disease with variable severity and medical presentations. Pediatric individuals have medical manifestations much like adults, but with different frequencies of organ involvement . These 2 instances demonstrate some of these variations. The most common medical manifestations of child years GPA at disease onset are related to top airway involvement (82%), nephropathy (65%), lower respiratory tract disease (61%), and musculoskeletal (55%). Nonspecific systemic symptoms such as fever and fatigue are also frequent (73%) [4, 7]. Case 2 investigation was initially prompted by nonspecific musculoskeletal symptoms, despite that additional findings were quickly found out. Case 1 demonstration was pneumonia-like, with systemic and respiratory symptoms. GPA severity of lung involvement is variable, TEMPOL ranging from asymptomatic pulmonary lesions, nodular lesions, or cavities to diffuse alveolar hemorrhage that can be fulminant and dramatically life threatening, as presented in Case 2 . On the other hand, it is also important to recall that GPA is definitely associated with a significative improved risk of thromboembolic events, such as pulmonary embolism and deep venous thrombosis, at disease demonstration and during follow-up, as explained in Case 1 [7, 8]. Active disease seems to present a major risk factor, and despite the pathogenetic background becoming poorly recognized, it TEMPOL appears to be related to endothelial function and integrity, induction of a state of hypercoagulability resulting from changes in pro- and anticoagulant factors, associated with an swelling status and eventually the use of cyclophosphamide and high doses of glucocorticoids [8C11]. At analysis, antineutrophil cytoplasmic antibodies (ANCA) play an essential TEMPOL role. They form a heterogeneous group of antibodies that target.