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Related Concept Videos

Acute Pyelonephritis II: Diagnostic Studies and Management01:28

Acute Pyelonephritis II: Diagnostic Studies and Management

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Introduction:For diagnosing acute pyelonephritis, a comprehensive patient history is collected to identify symptoms such as dysuria, frequent or urgent urination, flank pain, or costovertebral angle (CVA) tenderness that may suggest a kidney infection.Physical ExaminationDuring the physical examination, CVA tenderness is assessed. This involves gentle percussion over the costovertebral angle, where tenderness often indicates a kidney infection.Diagnostic TestsUrinalysis: Used to identify white...
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Acute Pyelonephritis I: Introduction01:27

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Pyelonephritis is a bacterial infection that primarily affects the renal parenchyma and collecting system, including the renal pelvis, tubules, and interstitial tissue of one or both kidneys. It can be classified as either acute—a sudden, severe infection—or chronic, which refers to long-term or recurrent kidney infections.The primary cause of acute pyelonephritis (APN) is bacterial infection, with Escherichia coli accounting for approximately 70-80% of cases. Other bacteria, such...
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Granulomatous polyangiitis with pachymeningitis: a case report.

Whei Chuern Yeoh1, Cristina Estrach2, Jagdish Nair2

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Granulomatosis with polyangiitis (GPA) can affect the nervous system and lungs. Early diagnosis and combination therapy including rituximab and avacopan are crucial for remission in severe GPA cases.

Keywords:
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Area of Science:

  • Neurology
  • Rheumatology
  • Pulmonology

Background:

  • Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis.
  • GPA can present with diverse neurological and systemic manifestations.
  • Cranial neuropathies and pachymeningitis are uncommon but serious GPA complications.

Purpose of the Study:

  • To report a case of GPA presenting with complex neurological and systemic involvement.
  • To highlight the diagnostic challenges and treatment strategies for GPA.
  • To evaluate the efficacy of novel therapeutic agents in refractory GPA.

Main Methods:

  • Case presentation of a 33-year-old male with headache and cranial neuropathies.
  • Diagnostic workup including MRI, CSF analysis, meningeal biopsy, and CT chest.
  • Treatment with cyclophosphamide, corticosteroids, rituximab, and avacopan.

Main Results:

  • Diagnosis of GPA confirmed by biopsy, with skull base mass, pachymeningitis, sigmoid sinus thrombosis, and lung nodules.
  • Initial treatment with cyclophosphamide and corticosteroids led to alveolar hemorrhage and mononeuritis multiplex.
  • Subsequent treatment with rituximab and avacopan resulted in clinical improvement and remission.

Conclusions:

  • GPA requires a high index of suspicion for diverse presentations.
  • Combination therapy with rituximab and avacopan can be effective in severe or refractory GPA.
  • Multidisciplinary management is essential for optimizing outcomes in GPA.