Friedrich Wegener was the author of a comprehensive description of a disease that was named Wegener\'s granulomatosis. In 2010, its name was changed to granulomatosis with polyangiitis (GPA), the reason being that Wegener\'s links to Nazism were revealed. The research was conducted between May 2021 and August 2023 and was based on the historical method (understood as defining the subject of research, critical analysis of collected historical source materials, and historiographical synthesis) using the methods of direct and indirect inference ascribed to the historical method. Wegener and his wife were members of numerous Nazi organizations. He was rewarded for his stance by the German Reich. Together with his family, he lived in very comfortable conditions for the standards of the time, unattainable for the average person. He described himself as a frontline combatant in the Nazi invasion of Poland. Friedrich Wegener\'s links with Nazism were more extensive than has hitherto been known. The links involved his professional and private life. They testify to his membership in numerous Nazi organizations, as well as his active involvement in the activities of various institutions and communities of the German Reich and his overt expression of support for Hitler. Wegener\'s numerous accolades from the German Reich providing him with professional, material and personal benefits were proof of his links with the Nazi movement. Wegener\'s association with Nazism helped him to advance his career, and to attain a high social status and quality of life, both before the outbreak and during the Second World War.

Granulomatosis with polyangiitis (GPA), formerly known as Wegener\'s granulomatosis (WG), is a condition marked by necrotizing vasculitis of the small-medium vessels that results in necrotizing granulomatous inflammation. Splenic involvement in GPA is a potentially life-threatening consequence of connective tissue disease and is rarely described as the main presenting feature. We present a case of a patient with perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) who experienced spontaneous splenic rupture. A CT scan of the abdomen, an ANCA test, and a splenic biopsy were employed to identify ANCA-associated vasculitis (AAV) splenic rupture. Our patient\'s splenic rupture could be attributed to GPA. Nonetheless, since it may alter patient follow-up and therapy, a patient with spontaneous splenic rupture without an obvious explanation should be promptly evaluated for connective-tissue disease. This report highlights the intricacy and unpredictability of the clinical symptoms linked to AAV, as well as the possibility of misinterpreting them.

Here, we report a case of granulomatosis with polyangiitis presenting with unilateral parotid gland enlargement and later developed skin lesions on the lower extremities and abdomen. Although rare, salivary gland enlargement may be the presenting sign of Wegener\'s granulomatosis or other ANCA associated vasculitides.

To share a unique case of granulomatosis with polyangiitis (GPA) identified in a child with CNS involvement, specifically PRES (posterior reversible encephalopathy syndrome). Discuss this uncommon manifestation\'s clinical characteristics, diagnostic process, and treatment. We are currently discussing a 12-year-old female patient who presented with a chronic cough, shortness of breath, and a new-onset fever. Upon further examination, the patient was diagnosed with GPA, confirmed through positive cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA), a renal biopsy, and multiple lung cavitary lesions. During her hospitalization, the patient also experienced neurological symptoms, including a severe headache, blurred vision, loss of consciousness, and an abnormal neurological exam, which led to brain MR imaging. The imaging revealed evidence of small vessel vasculitis with confluent T2 hyper signal intensity of gray-white matter junctions in both parietooccipital and frontal lobes containing hemorrhagic components, suggesting Posterior reversible encephalopathy syndrome. This case of Wegener\'s granulomatosis is noteworthy due to its occurrence in a pediatric patient with CNS involvement, specifically (posterior reversible encephalopathy syndrome). This event highlights the importance of recognizing that autoimmune disorders can present infrequently in young patients. Diagnosing Wegener\'s granulomatosis can be challenging, particularly when the CNS is affected. However, when appropriate treatment is initiated promptly, favorable outcomes can be achieved, as evidenced by the patient\'s improved condition with the prednisolone, captopril, and Rituximab treatment plan. Further research is necessary to understand better the underlying pathophysiology and optimal management of CNS involvement in GPA, particularly in the pediatric population.

BACKGROUND: Ultrasonographic evaluation of fetal thymus size may be used to predict the adverse perinatal outcome in pregnant women with vasculitis.
OBJECTIVE: To compare fetal thymus size in pregnant women with vasculitis and healthy pregnant women and to evaluate whether fetal thymus size predicts the adverse perinatal outcome.
METHODS: Twenty-two pregnant women with previously diagnosed vasculitis, 18 of them with Behçet\'s disease, three with Takayasu arteritis, and one with Wegener\'s granulomatosis, were included in the case group. The control group comprised 66 healthy pregnant women whose gestational ages matched the case group. Thymic thoracic ratio (TTR) was measured to assess fetal thymus size in the view of three vessels and trachea.
RESULTS: In the case group, fetal TTR was significantly lower (0.32 ± 0.03 vs. 0.36 ± 0.02, p = < 0.001). Fetal TTR was significantly lower in those using prednisone than those not (p = .001) in the case group. There was no significant difference in fetal TTR between colchicine used and not used (p = .078) in the case group. Also, for the TTR, a sensitivity of 100% and a specificity of 92% were achieved with a cut-off value of 0.33 for predicting adverse perinatal outcomes.
CONCLUSIONS: The fetuses of pregnant women with maternal vasculitis had a smaller TTR. The small fetal thymus may alert clinicians to possible adverse perinatal outcomes and, with other supporting risk factors, may help predict adverse perinatal outcomes in pregnant women with vasculitis.

ABSTRAK Penyakit Granulomatosis Wegener merupakan suatu penyakit autoimun yang mengenai dinding pembuluh darah terutama pembuluh darah kecil dan sedang akibat adanya reaksi komplek imun. Sampai saat ini etiologi penyakit Granulomatosis Wegener belum diketahui secara pasti. Diagnosis Granulosis Wegener salah satunya adalah terdeteksinya antibodi cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), akan tetapi pemeriksaan ANCA negatif atau tidak ditemukan pada kasWegener\'s granulomatosis is an autoimmune disease that affects the walls of small and medium-sized blood vessels due to an immune complex reaction. Meanwhile, at present, the etiology of the disease is unknown with certainty. One of the diagnoses is the detection of cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), but a negative ANCA examination is very rare. Therefore, this is a case report of a 33-year-old man that complained of sores on both legs, which were difficult to heal. The patient also experienced joint pain, fever at night, weight loss, hair loss as well as recurrent nosebleeds with an unknown cause. Furthermore, the physical examination found a saddle nose and black spots from the right and left groin to the back of the legs. Multiple irregular ulcers with different sizes were also discovered in the region cruris and dorsum pedis. The laboratory examination results showed Hb of 8.7 g/dl, 130 mm/hour ESR. Based on peripheral blood smear, the patient was suspected to have hypochromic-microcytic anemia, which caused chronic process along with bleeding. The IF pattern was also speckled with a titer of 1:320, and the ANCA test was negative (-). Meanwhile, the results of routine urine examination found blood +4 macroscopically and observed leukocyturia 2-10 LPB and 8-21 LPB erythrocyturia microsopically. The Doppler ultrasound of the left inferior extremity revealed the swelling of the left pedis soft tissue with peripheral arteritis in the cutis lesion area. The Anatomical Pathology examination showed non-specific chronic inflammation in the cruris and pedis region. Subsequently, the patient was administered with wound debridement by a surgeon, packed red cell (PRC) transfusion, metylprednisolone mg, azathioprine, and cefixime. After the treatment, the nosebleed was no longer felt, the joint pain reduced, and the fever improved.us ini sangat jarang terjadi. Artikel ini membahas mengenai kasus laki-laki 33 tahun datang dengan keluhan luka pada kedua tungkai kaki yang sulit sembuh disertai nyeri pada sendi, demam pada malam hari, penurunan berat badan, rambut rontok dan mimisan berulang yang tidak diketahui penyebabnya. Pada pemeriksaan fisik ditemukan saddle nose, terdapat bercak kehitaman dari kedua pangkal paha kanan dan kiri sampai ke punggung kedua kaki.  Pada regio cruris dan dorsum pedis didapatkan adanya ulkus multiple, ukuran bervariasi, irregular. Hasil pemeriksaan laboratorium menunjukkan Hb 8,7 gr/dl, LED 130 mm/jam, Hapusan darah tepi: anemia hipokromik-mikrositik suspek et causa proses kronis bersamaan dengan proses perdarahan. ANA-IF pola speckled 1:320, ANCA tes hasil negatif (-). Hasil pemeriksaan urin rutin makroskopis darah +4, dan mikroskopis ditemukan leukosituria 2-10 LPB, eritrosituria 8-21 LPB. Hasil USG doppler pada regio ekstremitas inferior sinistra menunjukkan kesan: swelling jaringan lunak pedis sinistra dengan arteritis perifer pada area lesi kutis. Hasil pemeriksaan Patologi Anatomi menunjukkan peradangan kronis non spesifik et regio cruris dan pedis. Tatalaksana yang dilakukan debridement luka oleh spesialis bedah, transfusi pack red cell (PRC), metylprednisolon  mg, azathioprine, cefixime. Hasilnya mimisan sudah tidak dirasakan, nyeri sendi berkurang tetapi masih ada, dan demam membaik. Kata Kunci: Granulomatosis wegener, Vaskulitis autoimun, ANCA negatif  ABSTRACT Wegener\'s granulomatosis is an autoimmune disease that affects the walls of small and medium-sized blood vessels due to an immune complex reaction. Meanwhile, at present, the etiology of the disease is unknown with certainty. One of the diagnoses is the detection of cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), but a negative ANCA examination is very rare. Therefore, this is a case report of a 33-year-old man that complained of sores on both legs, which were difficult to heal. The patient also experienced joint pain, fever at night, weight loss, hair loss as well as recurrent nosebleeds with an unknown cause. Furthermore, the physical examination found a saddle nose and black spots from the right and left groin to the back of the legs. Multiple irregular ulcers with different sizes were also discovered in the region cruris and dorsum pedis. The laboratory examination results showed Hb of 8.7 g/dl, 130 mm/hour ESR. Based on peripheral blood smear, the patient was suspected to have hypochromic-microcytic anemia, which caused chronic process along with bleeding. The IF pattern was also speckled with a titer of 1:320, and the ANCA test was negative (-). Meanwhile, the results of routine urine examination found blood +4 macroscopically and observed leukocyturia 2-10 LPB and 8-21 LPB erythrocyturia microsopically. The Doppler ultrasound of the left inferior extremity revealed the swelling of the left pedis soft tissue with peripheral arteritis in the cutis lesion area. The Anatomical Pathology examination showed non-specific chronic inflammation in the cruris and pedis region. Subsequently, the patient was administered with wound debridement by a surgeon, packed red cell (PRC) transfusion, metylprednisolone mg, azathioprine, and cefixime. After the treatment, the nosebleed was no longer felt, the joint pain reduced, and the fever improved. Keywords: Wegener\'s granulomatosis, Autoimmune vasculitis, negative ANCA.

Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing vasculitis of small to medium-sized blood vessels. The typical presentation is the classic triad of upper airway, pulmonary, and renal involvement. However, it can rarely present with the involvement of a single organ system known as limited granulomatosis with polyangiitis. We present a case of a 53-year-old male with chronic rhinosinusitis as the only manifestation of limited GPA. The diagnosis was established incidentally based on biopsy findings from the paranasal sinuses obtained during functional endoscopic sinus surgery. Subsequent testing revealed a positive cytoplasmic antineutrophilic antibody. No evidence of systemic involvement was noted. Prednisone and azathioprine were initiated leading to significant improvement. Although upper respiratory tract involvement is common in GPA, it is rare for the condition to be limited to this organ system. Our case of limited GPA is distinct in that it represents a rare presentation of this already rare disease.

To describe the clinical features and management of patients with scleritis associated with granulomatosis with polyangiitis (GPA) at a tertiary eye care center in South India.
The clinical profile and management of patients presenting to a tertiary eye care center in South India with scleritis secondary to GPA from 2003 to 2021 were analyzed retrospectively. Scleritis was classified into anterior diffuse, nodular, and necrotizing scleritis with inflammation according to Watson and Hayreh\'s classification. Demographic characteristics, clinical features, anti-neutrophil cytoplasmic antibody (ANCA) positivity, treatment response, ocular complications, and status at the last follow-up were analyzed. Statistical analysis of data was performed using Microsoft Excel 2019.
Nineteen eyes of 17 patients (15 cytoplasmic staining ANCA [c-ANCA], two p-ANCA positive) were included. Fifteen eyes had necrotizing scleritis, two had diffuse anterior scleritis, and two had nodular scleritis. Remission was induced using a combination of steroids and cyclophosphamide or rituximab. Maintenance therapy was instituted using tapering steroids and immunosuppressants like cyclophosphamide, mycophenolate mofetil, methotrexate, or rituximab. Three eyes required a scleral patch graft. Fourteen patients had good anatomical and visual outcomes, and three were lost to follow-up.
GPA is a rare disease, while it is the most common ANCA-associated vasculitis with scleritis. As scleritis may be the presenting sign of the disease, ophthalmologists must be aware of the various features suggestive of GPA. GPA-associated scleritis can have a good prognosis when diagnosed promptly and managed aggressively in the acute stage, and remission is maintained with adequate systemic immunosuppression.

UNASSIGNED: Ocular and orbital involvement in Granulomatosis with polyangiitis (GPA) is common. GPA can lead to life and sight threatening complications due to necrosis and tissue melting.
UNASSIGNED: We report four cases presenting with ocular pain and redness for varied durations. One had diminution of vision. All of them had deep sectoral/diffuse congestion with one having scleral thinning. All were diagnosed with anterior necrotizing/non-necrotizing scleritis. One had associated penetrating ulcerative keratitis. Topical steroids and systemic non-steroidal anti-inflammatory drugs were started in all cases and rheumatology consultation was taken. Pertinent investigations were sent, and GPA was diagnosed. Intravenous immunosuppressive regimens and oral steroid were started and significant improvements were seen, preventing untoward complications.
UNASSIGNED: Scleritis could be manifesting feature of GPA so cautious history taking and evaluation is important. Management often requires multidisciplinary care and ocular features could be the reference guidelines to adjust dose of systemic medications of GPA.

Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are rare systemic necrotizing vasculitis. The national incidence and prevalence of GPA/MPA and patient mortality remain unknown in France. A real-life study using retrospective data from the French National Health Data System was set up to describe the epidemiology and demographic characteristics of hospitalized GPA and MPA patients, overall and by disease.
All adult patients (≥18 years of age) hospitalized for GPA (ICD-10 M31.3) or MPA (ICD-10 M31.7) between 01 and 01-2010 and 31-12-2017 and affiliated to the General health insurance Scheme (covering 76% of the French population) were included in this national retrospective observational study. Descriptive analyses, univariate and multivariable logistic models, Kaplan-Meier survival analysis, and Cox models were performed.
The study involved 4445 prevalent GPA patients (including 1578 incident patients) and 1833 prevalent MPA patients (878 incident patients). Distinction between GPA and MPA diagnosis could not be made for 303 patients (149 incident patients). In people aged over 20 years, the age-standardized incidence rates of GPA and MPA were 0.5 and 0.3/100,000 person-years, respectively and the age-standardized prevalence rates were 10 and 4/100,000 person-years, respectively. The standardized mortality ratios in GPA and MPA patients aged over 20 years were 2.0 and 2.7, respectively, and remained constant. Renal failure, pulmonary and urinary tract infections, as well as coronary disease were more frequent among MPA than GPA patients. One-year survival rates among GPA and MPA patients were 96% (95%CI 94%-97%) and 94% (92%-95%), respectively. Five-year survival rates among GPA and MPA patients were 81% (95% CI 79%-83%) and 72% (68%-75%), respectively. After adjusting for comorbidities, the risk of death was still higher in MPA (hazard ratio 1.26 [95%CI 1.06-1.50]) than in GPA patients.
Despite advances in the therapeutic management of patients, mortality rates are still high and stable over time, highlighting the need for improved management.