Vasculitides: Symptoms, Complaints, Signs

Because of the many different forms of vasculitides, symptoms can be very diverse.

The following symptoms and complaints may indicate anti-GBM (glomerular basement membrane) disease, formerly Goodpasture’s syndrome:

  • Acute renal failure in “rapid progressive glomerulonephritis (RPGN).”
  • Hemoptysis (coughing up blood)
  • Pulmorenal syndrome – combination of renal and pulmonary vasculitis (inflammation of the (mostly) arterial blood vessels in the kidneys and lungs) including necrotizing extracapillary proliferative glomerulonephritis (inflammation of the glomeruli (renal corpuscles) of the kidneys)

The following symptoms and complaints may indicate eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss syndrome (CSS):

Renal involvement is rare. The following symptoms and complaints may indicate granulomatosis with polyangiitis (GPA), formerly Wegener’s granulomatosis, (according to the EUVAS definition):

  • Fatigue
  • Acute dyspnea (shortness of breath)
  • General weakness
  • Anorexia (loss of appetite)
  • Arthralgia (joint pain)
  • Eyelid / lower leg edema (water retention).
  • Cephalgia (headache), due to hypertension (high blood pressure).
  • Chronic bloody-crusted rhinitis (inflammation of the nasal mucosa).
  • Chronic otitis media (inflammation of the middle ear).
  • Episcleritis (inflammation of the episclera (highly perfused, loose layer of connective tissue that separates the sclera (sclera) from the tunica conjunctiva (conjunctiva) at the anterior margin of the eye)).
  • Increased temperature / fever
  • Exophthalmos (protrusion of the eye from the orbit).
  • Gait unsteadiness
  • Weight loss
  • Glomerulonephritis (inflammation of the glomeruli (renal corpuscles)), possibly “rapid progressive” form.
  • Skin changes such as purpura (punctate hemorrhages) or necrosis (tissue death).
  • Hemoptysis (coughing up blood)
  • Hemorrhage (bleeding from the respiratory tract).
  • Mastoiditis (mastoid process inflammation; purulent inflammation of the bone behind the ear).
  • Fatigue
  • Myalgia (muscle pain)
  • Myositis (muscle inflammation)
  • Night sweats (night sweats)
  • Pharyngitis (inflammation of the throat)
  • Saddle nose
  • Visual disturbances
  • Septum perforation (hole in the nasal septum)
  • Sialadenitis (inflammation of the salivary glands)
  • Subglottic tracheal stenosis – tracheal narrowing below the glottis.
  • Numbness/sensitivity disorders, especially in fingers and tips of feet.
  • Ulcerations (ulcers) in the oropharynx (oral pharynx).

The following symptoms and complaints may indicate isolated leukocytoclastic cutaneous vasculitis:

  • Punctate redness of the skin; may confluence.
  • Blood blisters – bluish, often painful nodules.
  • Undefined reticular or stellate skin lesions.
  • Poorly healing ulcers (boils)

Skin lesions often occur on the legs. The following symptoms and complaints may indicate Kawasaki syndrome (MCLS):

  • Septic temperatures, lasting longer than five days; antibiotics have no effect.
  • Exanthema (rash) with scaling beginning in the second to third week, starting at the fingertips
  • Conjunctivitis (conjunctivitis), usually bilateral (occurring on both sides).
  • Brittle lacquer lips
  • Stomatitis (inflammation of the oral mucosa) with a strawberry tongue.
  • Cervical lymphadenopathy – enlargement of the lymph nodes of the neck; usually unilateral.
  • Palmar erythema – redness of the palms.
  • Erythema (areal redness of the skin) and edema (water retention) on the hands and feet.
  • Icterus (jaundice)

The following symptoms and complaints may indicate microscopic polyangiitis (MPA):

Other symptoms

  • Episcleritis (inflammation of the episclera (highly perfused, loose layer of connective tissue separating the sclera (sclera) from the tunica conjunctiva (conjunctiva) at the anterior margin of the eye)).
  • Myalgias (muscle pain) and arthralgias (joint pain).
  • Polyneuritis (inflammation of the nerves)
  • Sinusitis (sinusitis)

The following symptoms and complaints may indicate polyarteritis nodosa (PAN; panarteritis nodosa):

  • Cachexia (emaciation).
  • Fever
  • Abdominal pain (abdominal pain)
  • Circulatory disorders, unspecified
  • Iron deficiency anemia
  • Joint pain
  • Leukocytosis – increase in white blood cells.
  • Neuritis (inflammation of the nerves)
  • Nephropathy (kidney disease)

The following symptoms and complaints may indicate Schönlein-Henoch purpura:

  • Vomiting
  • Exanthema (skin rash)
  • Fever
  • Gastrointestinal bleeding – bleeding in the gastrointestinal tract.
  • Joint swelling, especially arthritis of the ankle joints.
  • Hematuria – blood in the urine
  • Colicky abdominal pain/agina abdominalis (intestinal colic) (100% of cases).
  • Headache
  • Palpable petechiae/palpable purpura (multitude of pinhead-sized hemorrhages from capillaries into the skin or mucous membranes; preferred region: legs and buttocks)
  • Proteinuria – increased excretion of protein with urine.
  • Severe feeling of illness
  • Behavioral disturbances