Polyarteritis Nodosa (PAN)

By SWA

Definition

Polyarteritis Nodosa (PAN) is a rare, systemic necrotizing vasculitis that predominantly affects medium-sized muscular arteries, leading to organ damage and a wide array of clinical manifestations.

Etiology

The exact cause of PAN is unknown, but it is believed to be associated with immune system dysfunction. In some cases, it has been linked to hepatitis B or C infections.

Pathophysiology

The disease involves inflammation of the arterial walls, leading to thickening, weakening, and necrosis of the vessel walls. This process can cause aneurysms, thrombosis, and subsequent organ ischemia.

Clinical Features

  • Systemic: Fever, weight loss, malaise
  • Cardiovascular: Hypertension
  • Renal: Renal infarcts, hypertension
  • Gastrointestinal: Abdominal pain, gastrointestinal bleeding
  • Neurological: Mononeuritis multiplex, peripheral neuropathy
  • Dermatological: Livedo reticularis, nodules, ulcerations

Diagnosis

  • Clinical examination and history
  • Laboratory tests: Elevated inflammatory markers (ESR, CRP), hepatitis B and C serologies
  • Imaging: Angiography can reveal aneurysms and stenosis in medium-sized arteries
  • Biopsy: Demonstrating necrotizing vasculitis in affected tissue

Treatment

  • Medications: Corticosteroids and immunosuppressive agents (e.g., cyclophosphamide)
  • Antiviral therapy: If associated with hepatitis B or C
  • Supportive care: For organ-specific complications

Livedoid Vasculopathy

Definition

Livedoid vasculopathy (LV) is a chronic, recurrent thrombotic condition affecting the small blood vessels, typically presenting with painful ulcers on the lower extremities.

Etiology

The precise cause is unclear, but it is thought to be related to abnormalities in blood coagulation and fibrinolysis, leading to thrombotic occlusions in the small blood vessels.

Pathophysiology

LV involves occlusion of small dermal blood vessels by fibrin thrombi, resulting in ischemia and subsequent ulceration. It is considered a thrombotic microangiopathy rather than a primary vasculitis.

Clinical Features

  • Ulcers: Painful, recurring ulcers on the lower legs, especially around the ankles
  • Scarring: Atrophic white scars (atrophie blanche) with surrounding hyperpigmentation
  • Discoloration: Livedo reticularis (a net-like pattern of reddish-blue skin discoloration)

Diagnosis

  • Clinical examination and history
  • Biopsy: Affected skin showing thrombotic occlusion of small vessels with minimal inflammation
  • Laboratory tests: To rule out underlying hypercoagulable states

Treatment

  • Medications: Antiplatelet or anticoagulant therapy (e.g., aspirin, warfarin)
  • Vasodilators: To improve blood flow
  • Pain management
  • Wound care: For ulcers

Cryoglobulinemic Vasculitis

Definition

Cryoglobulinemic vasculitis is a type of vasculitis characterized by the presence of cryoglobulins—proteins that precipitate in the cold and cause inflammation and damage to blood vessels.

Etiology

Often associated with chronic infections (e.g., hepatitis C), autoimmune diseases (e.g., systemic lupus erythematosus), and hematologic malignancies (e.g., multiple myeloma).

Pathophysiology

Cryoglobulins precipitate at low temperatures and deposit in small to medium-sized blood vessels, leading to complement activation, inflammation, and vascular damage.

Clinical Features

  • Dermatological: Palpable purpura (small, raised areas of bleeding under the skin)
  • Musculoskeletal: Arthralgia (joint pain)
  • Renal: Glomerulonephritis (kidney inflammation)
  • Neurological: Peripheral neuropathy
  • Systemic: Fatigue, fever, weight loss

Diagnosis

  • Detection of cryoglobulins in the blood
  • Serum protein electrophoresis and immunofixation: To characterize cryoglobulins
  • Complement levels: Often decreased
  • Biopsy: Affected tissue showing vasculitis with cryoglobulin deposits

Treatment

  • Antiviral therapy: For hepatitis C
  • Immunosuppressive therapy: E.g., corticosteroids, rituximab
  • Plasmapheresis: In severe cases
  • Treatment of underlying conditions: E.g., malignancies, autoimmune diseases

Each of these conditions involves distinct mechanisms and treatment approaches, emphasizing the importance of accurate diagnosis and tailored therapy to manage the specific disease manifestations effectively.


 

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