The Vasculitides

© Dirk Biddle

1.4 Clinical Features - Signs and Symptoms

The signs and symptoms of vasculitis conditions are various and depend on the type of vasculitis and on the particular blood vessels involved. However, general constitutional symptoms most often associated with the vasculitides include fever, fatigue, malaise, night sweats, weight loss, rapid pulse, and diffuse or acute pain. Indeed, vasculitis has been noted as a “hurting disease” because it is so commonly associated with pain of one type or other (1) - pain from nerve infarction (nerves often follow blood vessels so inflammation in blood vessels can also lead to nerve damage), pain from insufficient blood or oxygen to the kidneys, gastrointestinal tract or other organs, pain from skin ulcers, muscle pain (myalgia) - but identifying the source of pain can often be challenging, as vasculitis conditions are known to affect virtually every organ system in the body and often pain from an organ is referred to, or experienced in, another part of the body.

Generally a person suffering a primary vasculitis condition will experience a variety of symptoms that either singly or together come and go over time. Initially these symptoms may often be dismissed as not particularly serious, or mistaken for a manifestation of other conditions, before an acute onset of one or other of the symptoms, which may include hallmarks of vasculitis conditions; include renal dysfunction (glomerulonephritis,infarction, skin rashes (palpable purpura) and neurologic involvement (pins and needles, tingling, weakness and numbness, and foot - or wrist-drop(Foot-drop: Paralysis or weakness of the dorsiflexor muscles of the foot, as a consequence of which the foot falls, the toes dragging on the ground in walking; many causes, both central and peripheral. (OMD))Wrist-drop: Paralysis of the extensors of the wrist and fingers; most often caused by lesion of the radial nerve. (OMD)) -may lead to a more accurate diagnosis. Indeed, a common feature of vasculitis conditions seems to be the difficulty that patients often describe in obtaining an accurate and swift diagnosis from medical practitioners; often presenting themselves over many months or sometimes years before a correct diagnosis is obtained. It is also probable that many people may develop subacute (latent) or mild vasculitis symptomatology without it ever being specifically associated with a vasculitis condition. More rarely an acute onset of primary vasculitis may be preceded by no noticeable symptomatology at all, such as in Polymyalgia rheumatica where patients often describe retiring in the evening feeling well but waking the next morning inexplicably suffering neck and shoulder pain, often also involving the hip area.

Secondary vasculitides are usually preceded by a disease process that will have its own symptomatology and this may complicate the presentation of any symptomatology that may be due to a subsequent or comorbid vasculitis condition.

A vasculitis condition manifesting in a specific organ system may present with specific symptomatology related to that organ, but may also present with symptomatology seemingly dislocated from the actual site of pathology. In some cases a vasculitis will be limited to a specific organ, such as in retinal vasculitis or cutaneous vasculitis - and here symptom presentation will often depend on the severity of inflamation - in other cases multiple organs or organ systems might be invloved. As indicated above, there will be some who experience vasculitis conditions on a subacute level, there will be others however unfortunate enough to experience a range of serious and sometimes fatal conditions. The following list will provide the reader a sense of the almost bewildering extent and range of symptomatology presented by the vasculitides (the list is neither complete nor definitive). Here the reader is encouraged to utilise the glossary for particular definitions of the terms used.

Organ systems commonly involved in vasculitis conditions may include:

Central nervous system:

Eye, ear, & nose

Gastrointestinal tract

Musculoskeletal system:

Peripheral arterial system:

Peripheral nervous system:

Respiratory tract:

Skin:

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a) infarction (The formation of an infarct. (OMD))

b) myalgia(Pain in a muscle or muscles. (OMD))

c) glomerulonephritis (A variety of nephritis characterised by inflammation of the capillary loops in the glomeruli of the kidney. It occurs in acute, subacute and chronic forms and may be secondary to haemolytic streptococcal infection. Evidence also supports possible immune or autoimmune mechanisms. (OMD))

d) purpura A small haemorrhage (up to about 1 cm in diameter) in the skin, mucous membrane or serosal surface, which may be caused by various factors, including blood disorders, vascular abnormalities and trauma. (OMD)) Thus palpable purpura- purpura that are able to be 'palpated' or felt. Small haemorrhages are classified according to size as petechiae (very small), purpura (up to 1 cm) and ecchymoses (larger). The massive accumulation of blood within a tissue is called a haematoma.(OMD)), necrotic ulcers(Necrosis:The sum of the morphological changes indicative of cell death and caused by the progressive degradative action of enzymes; it may affect groups of cells or part of a structure or an organ. (OMD)

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1. Johns Hopkins Vasculitis Center. (1998) What is Vasculitis? (http://vasculitis.med.jhu.edu/whatis/symptoms.html).