Symptoms of Vasculitis

What are the symptoms of vasculitis?

The symptoms of vasculitis depend on the particular blood vessels that are involved by the inflammatory process.

Different types of vasculitis involve blood vessels in characteristic locations throughout the body. For example, Giant Cell Arteritis typically involves the medium– to large–sized blood vessels supplying the head and neck, but rarely involves the blood vessels of the kidneys. In contrast, Wegener’s Granulomatosis (GPA) frequently involves the kidneys, very often the lungs, and almost always the upper respiratory tract, but rarely blood vessels to the brain. As depicted in the image below, Buerger’s disease involves the fingers and (toes). Gangrene can result from a profound lack of blood flow.

buergerslg

Different types of vasculitis have characteristic (localized) patterns of blood vessel involvement.  However, vasculitis is a systemic illness. Thus, patients with vasculitis feel sick. They often have fevers, weight loss, fatigue, a rapid pulse, and diffuse aches and pains that are difficult to pinpoint. It has been said that vasculitis is a “hurting disease”, because it is so commonly associated with pain of one type or another: pain from a nerve infarction, pain from insufficient blood to the gastrointestinal tract, pain from skin ulcers. In some cases, however, identifying the source and underlying cause of the pain is extremely challenging.  In addition to these diffuse, poorly–localized “constitutional symptoms”, vasculitis may involve virtually every organ system in the body.

What organ systems may be affected?

It is important to note that not every organ system will be affected in every patient. The pattern of organ involvement (and symptoms) is unique to the individual, as well as the type of vasculitis (category).

A variety of rashes, the most classic of which is “palpable purpura” –purplish–red spots, usually found on the legs. These spots can usually be felt by the examiner’s fingertips, hence the descriptor “palpable”. The image below is a classic example of palpable purpura. These lesions result from the leakage of blood into the skin through inflamed, damaged blood vessels. They tend to occur in “crops”.

Purpura

Repeated bouts of purpura may lead to hyperpigmented areas of the skin, as shown below.

Skin

Symptoms range from full–blown arthritis to aches in the joints without obvious swelling (arthralgias).

Pictured below is an example of Henoch-Schönlein purpura: cutaneous vasculitis manifested by palpable purpura and arthritis (note the right ankle swelling). The diagnosis was confirmed by a skin biopsy, with immunofluorescence positive for IgA deposition witin blood vessel walls.

Leurpura

Cough (particularly coughing up blood), shortness of breath, a pneumonia–like appearance to a patient’s chest X–ray, lung “infiltrates”, and the development of cavities in the lungs.

Below is an image of a CAT scan of the lungs of a 73 year–old woman complaining of constitutional symptoms, shortness of breath, and bloody sputum. The patient also had glomerulonephritis, a positive P–ANCA, and antibodies to myeloperoxidase. The diagnosis of microscopic polyangiitis was made.

Lungs

Eleven days later, as the patient’s symptoms worsened, a chest X–ray confirmed progression of her lung hemorrhage. The X–ray shows fluffy infiltrates in both lungs, representing bleeding from damaged capillaries.

Chest Xray

Red blood cells (usually invisible to the naked eye), clumps of red blood cells (known as “casts”, also invisible to the naked eye), and loss of protein in the urine. May lead to renal insufficiency and the requirement of dialysis. Depicted in the figure below is a single glomerulus (the filtering unit of the kidneys; each kidney has approximately 1 million glomeruli). This glomerulus is involved actively by an inflammatory process, particularly evident in the bottom of the figure.

Glomerulus

Abdominal pain, bloody diarrhea, perforation of the intestines.

angiogram

Anemia (low hematocrit or red blood cell count) and/or a slightly elevated white blood cell count.

Chronic sinus congestion and “infections” that persist for longer than they should; hearing loss; inflammation of the nasal septum, sometimes resulting in a perforation or collapse of the bridge of the nose, as shown in the picture below.

Nose

May affect either blood vessels to the eyes, causing the sudden loss of vision, or small blood vessels withinthe eyes, leading to retinal problems, thinning of the sclera (the white part of the eyes), inflammation within the eye’s different chambers, and conjunctivitis (“pinkeye”). Pictured below is an example of retinal vasculitis in a patient with systemic lupus erythematosus (lupus). The white areas represent regions of retinal infarction caused by vasculitis.

arterieslg

Headaches, strokes, changes in mental status, difficulty with coordination. Below, a magnetic resonance (MR) imaging study of the brain in central nervous system vasculitis demonstrates an intra–cerebral hemorrhage (bright area).

brainhemlg

Shooting pains in the arms and legs, numbness, and asymmetrical weakness (i.e., weakness that involves one side of the body more than the other).

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