wegener’s granulomatosis
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2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zohreh Rostami ◽  
Luca Cegolon ◽  
Nematollah Jonaidi Jafari ◽  
Nasrin Gholami ◽  
Seyed Vahid Mousavi ◽  
...  

Abstract Background Granulomatosis with polyangiitis (GPA), also known as Wegener’s granulomatosis, is an idiopathic systemic disease typically affecting the lungs, although other organs may also be involved. Case presentation A 28-year-old male was admitted to Baqiyatallah university hospital in Teheran (Iran) after a 3-week history of fever and productive cough. The patient gradually developed fatigue, arthralgia, hematuria, nausea, vomiting, dyspnea, hemoptysis, weight loss, oliguria and then anuria. Chest-X-ray (CXR) and computerized tomography scan revealed cavitating nodular opacities in the right lung lobe. Furthermore, plasma creatinine increased from 2.2 to 4 mg/dl in a few days. Histopathological examination of kidney biopsy revealed peri-glomerular and peri-vascular inflammation, degeneration and necrosis of the tubular epithelial lining, red blood cell casts, distorted glomerular structure, fibrin thrombi, segmental breaks of the glomerular basal membrane, disruption of Bowman's capsular membrane and crescent formation of the affected glomeruli. An abnormal CXR, an abnormal urinary sediment and a typical kidney histology were used as criteria to diagnose glomerulonefritis with poliangiitis (GPA). Bronchoalveolar lavage smear and PCR turned out positive for mycobacterium tuberculosis. After 3 months of treatment for (GPA) and tuberculosis the patient developed headache and seizure. Cerebral Magnetic Resonance Venography revealed cerebral venous thrombosis of the sinus transverse and sigmoid. Conclusions Tuberculosis may coexist with GPA, as it occurred in our patient. Since a crescentic glomerulonephritis can progress to renal failure, clinicians should always be aware of potential multiple conditions when considering differential diagnoses.


2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Xiaojing Gu ◽  
Liqiong Ma ◽  
Minghai Shi ◽  
Shuhong Chi ◽  
LiYa Huang

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
A. Gravos ◽  
K. Katsifa ◽  
P. Tselioti ◽  
V. Grammatikopoulou ◽  
K. Sakellaridis ◽  
...  

Abstract Background Aneurysm formation is a possible, but rare, complication of granulomatosis with polyangiitis, known as Wegener’s granulomatosis. Urgent diagnosis and therapy is very important because a ruptured aneurysm could be life threatening. Case presentation We, therefore, present the case of a 63-year-old Greek man who was diagnosed with granulomatosis with polyangiitis and retroperitoneal hematoma due to ruptured aneurysm in renal artery and upper pancreaticoduodenal artery. His clinical course was complicated by acute renal failure and acute respiratory failure due to alveolar hemorrhage. Emergency coil embolization was performed. Postembolization recovery was uneventful; no bleeding occurred. The patient underwent mechanical ventilation and continuous veno-venous hemofiltration and received combined immunosuppression and supportive therapy, but eventually died 30 days after admission to hospital from severe septic shock and multiple organ failure. Conclusion Endovascular treatment is the therapy of choice, especially for patients with ruptured aneurysms that are hemodynamically stable. Early diagnosis is very important, as urgent embolization and early initiation of immunosuppression therapy are the treatment of choice.


2021 ◽  
Vol 9 (6) ◽  
Author(s):  
Jesús Rojas‐Jaimes ◽  
Víctor Hugo Rojas‐Figueroa ◽  
Rodrigo Corcuera ◽  
José Arenas ◽  
Julio García‐Reynoso

2021 ◽  
Vol 65 (1) ◽  
pp. 93-97
Author(s):  
Andrada-Elena Mirescu ◽  
Ioana Teodora Tofolean ◽  
Mihaela Florica Milicescu ◽  
Irina-Elena Cristescu ◽  
Andrei Teodor Iacob ◽  
...  

2021 ◽  
Vol 12 (2) ◽  
pp. 309-313
Author(s):  
Bryan Arief Aji Rudita ◽  
Yulyani Werdiningsih ◽  
Arief Nurudhin ◽  
Nurhasan Agung Prabowo ◽  
Zainal Arifin Adnan

A B S T R A C TBackground: Wegener's granulomatosis is a very rare long-term systemicdisorder, in which granuloma formation occurs and inflammation of bloodvessels (vasculitis). The cause of disorder is not yet known, but geneticfactors are thought to play an important role. Clinical symptoms are oftensimilar to other disorders, making diagnosis difficult. However, earlydiagnosis is very important in order to provide effective management.Objective: Diagnosis and management in a rare case of Wegener’sgranulomatosis vasculitis, especially it was found with other comorbidities.Methods: This case report showed a-38-years-old-man that came to theemergency room of Dr Moewardi Hospital with complaints of swellingaccompanied by redness and stiff on the face, hands and feet that worseningsince 7 days ago. He also complained of fever fluctuating, nasal congestionaccompanied by clear discharge and sometimes hearing loss in the rightear. Since the last 3 months, he was often experience similar complaints.History of diabetes was recognized by the patient for 5 years, but he did notregularly take medication. Results: In this case, examination of vital signswithin normal limits. Physical examination revealed a saddle nose with cleardischarge, swelling and redness around the face, hands and feet. Laboratorytests showed hemoglobin 12.9 g/dl, HbA1c 8.4%, sodium level 128 mmol/L,potassium level 3.1 mmol/L, calcium level 1.12 mmol/L. The Anti -Neutrophil Cytoplasmic Antibodies (ANCAs) and Anti Nuclear Antibody(ANA) Indirect Immunofluorescence (IF) method were negative.Electrocardiogram and chest x-ray examination within normal limits.Histopathological examination revealed epidermal atrophy and multiplegranulomas of the dermis. The patient underwent treatment for 10 dayswith tappering-off dose steroid, immunosuppressants, insulin, calcium,and potassium preparations therapy. Conclusions: Wegener’sgranulomatosis vasculitis is a rare case. Prompt and accurate diagnosis andmanagement will prevent poor progression of them, especially it was foundwith other comorbidities.


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