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(MPA) appeared in the European literature in the 1920s. The concept of this disease as a
condition that is separate from polyarteritis nodosa (PAN) and other forms of vasculitis did
not begin to take root in medical thinking, however, until the late 1940s. Even today, some
confusing terms for MPA (e.g., microscopic poly arteritis nodosa rather than microscopic
poly angiitis ) persist in the medical literature. Confusion regarding the proper nomenclature
of this disease led to references to microscopic polyarteritis nodosa and hypersensitivity
vasculitis for many years. In 1994, The Chapel Hill Consensus Conference recognized MPA
as its own entity, distinguishing it in a classification scheme clearly from PAN, Wegeners
Granulomatosis (WG), cutaneous leukocytoclastic angiitis (CLA), and other diseases with
which MPA has been confused with through the years.
Much of the explanation for the difficulty in separating MPA from other forms of vasculitis
has stemmed from the numerous areas of overlap of MPA with other diseases. MPA, PAN,
WG, CLA, the Churg-Strauss syndrome, and other disorders all share a variety of features but
possess sufficient differences as to justify separate classifications.
Kidney Inflammation
Inflammation in the kidneys, known as glomerulonephritis, causes blood and protein loss
through the urine. This process can occur either slowly or very rapidly in the course of the
disease. Patients with kidney inflammation may experience fatigue, shortness of breath, and
swelling of the legs.
The image below is from a urinalysis of a patient with kidney inflammation. When MPA is
active, red blood cells will form a clump or cast (bracketed in white) within the tubules of
inflamed kidneys. These casts pass through the renal system and may be viewed under the
microscope in a patients urine.
Constitutional Symptoms
Weight loss, fevers, fatigue, and malaise are part of a collection of complaints regarded as
constitutional symptoms. Constitutional complaints are a common finding in patients with
MPA, because the disorder is a systemic disease confining itself generally not to one specific
organ system but rather broadly affecting a patients constitution.
Skin lesions
Skin lesions in MPA, as in other forms of vasculitis that involve the skin, can erupt on various
areas of the body. The lesions tend to favor the dependent areas of the body, specifically the
feet, lower legs and, in bed-ridden patients, the buttocks. The skin findings of cutaneous MPA
include purplish bumps and spots pictured below (palpable purpura).
These areas range in size from several millimeters in diameter to coalescent lesions that are
even larger. Skin findings in MPA may also include small flesh-colored bumps (papules);
small-to-medium sized blisters (vesiculobullous lesions); or as small areas of bleeding under
the nails that look like splinters (pictured below), hence the name splinter hemorrhages.
Pictured:
The hand on the left (the patients right hand) is normal, displaying normal muscle bulk of the
areas between the fingers. In contrast, the hand on the right (the patients left) shows wasting
of the muscle in the web space between the thumb and first finger, leading to a hollowed-out,
bowl-like appearance of that area. The consequence of this muscle wasting is that the patient
is unable to grasp objects between his thumb and fingers (i.e., has a weak pinch) and his hand
grip is weak.
Lungs
Lung involvement can be a dramatic and life-threatening manifestation of MPA. When lung
disease takes the form alveolar hemorrhage bleeding from the small capillaries that are in
contact with the lungs microscopic air sacs the condition may quickly pose a threat to the
patients respiratory status (and therefore to the patients life). Alveolar hemorrhage (pictured
below), which is frequently heralded by the coughing up of blood, occurs in approximately
12% of patients with MPA .
Organs that also merit mention in discussions of MPA include the eyes, muscles, and joints.
Intermittent irritation of the eye (resembling pinkeye) that is caused by either conjunctivitis
or episcleritis may be an early disease manifestation or a sign of a disease flare. Occasionally
other types of inflammation (e.g., uveitis) are also observed in MPA. Muscle or joint pains
(known to clinicians as myalgias or arthralgias, respectively) are common complaints in
MPA, generally accompanying the types of constitutional symptoms mentioned above.
Arthritis (inflammation of the joints accompanied by swelling) can also be observed in MPA.
Joint complaints in MPA and related forms of vasculitis tend to migrate from one joint to
another one day involving the left ankle, the next day the right wrist, the third day a
shoulder, for example.
GRANULOMATOUS
INFLAMMATION
LUNG SYMPTOMS
GLOMERULONEPHRITIS
RENAL HYPERTENSION
MONONEURITIS MULTIPLEX
SKIN LESIONS
GI SYMPTOMS
EYE SYMPTOMS
ANCA-POSITIVITY
CONSTITUTIONALSYMPTOMS
NECROTIZING TISSUE
MICROANEURYSMS
MPA
Small to Medium
Arterioles to
venules, And
sometimes Arteries
and veins
NO
WG
Small to Medium
Arterioles to venules,
And sometimes
Arteries and veins
PAN
Medium
Muscular
Arteries
YES
NO
YES1
YES
NO
COMMON
YES2
NO
YES4
75%
YES5
YES
RARELY
YES1
YES
NO
OCCASIONAL
YES2
NO
YES4
65-90%
YES5
YES
RARELY
NO
NO
YES
COMMON
YES2
YES3
NO
NO
YES5
YES
TYPICAL
MPA can have small blood vessel skin lesions as mentioned above, similar to WG or
medium blood vessel lesions similar to PAN (livedo reticularis, nodules, ulcers, and digital
gangrene)
3
MPA eye complications are typically milder than those of WG, but serious
Constitutional symptoms include weight loss, fevers, joint and muscle aches, and malaise.