Related Experiment Videos

[A case of Wegener's granulomatosis with pachymeningitis]

Mio Hayakawa1, Masayoshi Nishijima, Kyoko Inui

  • 1Respiratory Center, Yodogawa Christian Hospital.

Nihon Kokyuki Gakkai Zasshi = the Journal of the Japanese Respiratory Society
|May 22, 2009
PubMed

Insights

Pachymeningitis, inflammation of the brain

Area of Science:

  • Neurology
  • Rheumatology
  • Pathology

Background:

  • Wegener's granulomatosis (WG), a systemic vasculitis, can affect multiple organs.
  • Pachymeningitis, inflammation of the dura mater, is a rare neurological complication of WG.

Observation:

  • A 49-year-old male presented with headache, rhinorrhea, fatigue, bilateral lung nodules, and positive C-ANCA.
  • Brain MRI revealed pachymeningitis; WG was confirmed via sinus and kidney biopsies.
  • Treatment with corticosteroids and cyclophosphamide led to significant symptom improvement.

Findings:

  • A literature review identified only 15 previously reported cases of WG with pachymeningitis in Japan.
  • In some WG cases, pachymeningitis preceded systemic symptoms and was C-ANCA negative.
  • Pathological examination of pachymeningitis in WG predominantly showed necrotizing granulomas, suggesting granulomatous inflammation rather than vasculitis.

Implications:

  • Pachymeningitis in WG may represent an extension of upper respiratory tract granulomatous inflammation.
  • This finding suggests a distinct pathological mechanism for WG-associated pachymeningitis, potentially involving granuloma formation over angitis.
  • Further research is warranted to elucidate the pathogenesis and optimal management of this rare WG complication.

Related Concept Videos

Bacterial Meningitis I: Introduction01:22

Bacterial Meningitis I: Introduction

Bacterial meningitis is a severe, life-threatening inflammation of the meninges, particularly the pia mater and arachnoid mater, affecting the subarachnoid space, ventricles, and cerebrospinal fluid (CSF). If untreated, it can lead to significant neurological complications or death.Causative AgentsCommon pathogens vary with age and immune status. In adults, major organisms include Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. Streptococcus agalactiae (group B...
Bacterial Meningitis01:24

Bacterial Meningitis

Bacterial meningitis is a severe infectious disease involving inflammation of the meninges, the protective membranes surrounding the brain and spinal cord. It occurs when pathogenic bacteria cross the blood–brain barrier and enter the cerebrospinal fluid. Common causative organisms include Neisseria meningitidis, Streptococcus pneumoniae, Haemophilus influenzae type b, Listeria monocytogenes, and Escherichia coli K1. The exact route of entry varies by pathogen and host condition.Routes of Entry...
Bacterial Meningitis II: Pathophysiology01:26

Bacterial Meningitis II: Pathophysiology

Bacterial meningitis typically begins when pathogens such as Neisseria meningitidis and Streptococcus pneumoniae colonize the nasopharynx and invade the bloodstream. This process is facilitated by bacterial virulence factors, such as polysaccharide capsules, which resist phagocytosis and complement-mediated killing. Less commonly, bacteria reach the central nervous system via contiguous spread from infections like otitis media or sinusitis, through congenital or acquired dural defects, or...
Cryptococcal Meningitis01:27

Cryptococcal Meningitis

Cryptococcal meningitis is a life-threatening opportunistic infection predominantly associated with HIV/AIDS, accounting for over 100,000 deaths annually worldwide. However, it also affects individuals with other forms of immunosuppression, including those undergoing immunosuppressive therapy, organ transplant recipients, patients with innate immunodeficiencies, and individuals with hematological disorders. The infection is caused mainly by Cryptococcus neoformans and Cryptococcus gattii,...
Brain Abscess l: Introduction01:26

Brain Abscess l: Introduction

A brain abscess is a focal, intracerebral infection characterized by a localized collection of pus within the brain parenchyma, resulting from microbial invasion and the body’s inflammatory response. It progresses through stages: early and late cerebritis, followed by early and late capsule formation, reflecting tissue destruction, immune response, and eventual encapsulation.Etiology and PathogenesisCausative organisms vary with source and host factors, often involving polymicrobial infections,...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...