1124
U N I T 1 2
Musculoskeletal Function
blood cells and platelets
43,45
(Fig. 44-7). The onset may
be acute or insidious, and the course of the disease is
characterized by exacerbations and remissions.
Arthralgias and arthritis, the most common manifes-
tations of SLE, are present in up to 90% of persons at
some point during the course of their disease.
46
Although
the arthritis can affect any joint, it is most often sym-
metric with involvement of the small joints of the hands,
wrists, and knees. Ligaments, tendons, and the joint cap-
sule may be involved, causing varied deformities. Other
musculoskeletal manifestations of SLE include tenosyno-
vitis, rupture of the intrapatellar and Achilles tendons,
and avascular necrosis, frequently of the femoral head.
Skin manifestations can vary greatly and may be
classified as acute, subacute, or chronic. The acute skin
lesions include the classic malar or “butterfly” rash
on the nose and cheeks
43,45
(see Fig. 44-7). Other skin
lesions that may occur include hives or livedo reticularis
(i.e., reticular cyanotic discoloration of the skin, often
precipitated by cold), and fingertip lesions. Hair loss is
common. Mucous membrane lesions tend to occur dur-
ing periods of exacerbation. Sun sensitivity may occur in
SLE even after mild exposure.
Renal involvement is common in SLE and a signifi-
cant cause of morbidity and mortality.
45
Several forms
of renal involvement may occur, including glomerulone-
phritis, tubulointerstitial nephritis, and vascular disease
(see Chapter 25). The clinical manifestations of renal
involvement range from asymptomatic hematuria and/or
protinuria to frank nephrotic syndrome with progressive
loss of renal function. Nephrotic syndrome causes pro-
teinuria with resultant edema in the legs and abdomen,
and around the eyes. Kidney biopsy is the best determi-
nant of renal damage and the type of treatment needed.
Pulmonary involvement is manifested primarily by
pleural effusions and/or pleuritis.
45
Pleural effusions are
typically small, bilateral, and exudative. Up to 50% of
persons with SLE develop pleuritis, which is manifested
by pleuritic chest pain. The presence of pleuritis usually
corresponds to active SLE in other organ systems.
Cardiovascular disease is a frequent complication
of SLE. It can involve the pericardium, myocardium,
or coronary arteries. Pericarditis, with or without effu-
sion, is the most common cardiac manifestation in SLE,
occurring in 50% of persons with SLE at some point
during the course of their disease.
45
Coronary heart dis-
ease is also increased in persons with SLE. Hypertension
may be associated with lupus nephritis and long-term
corticosteroid use. Hematologic disorders may mani-
fest as hemolytic anemia, leukopenia, lymphopenia, or
thrombocytopenia.
The nervous system is involved in persons with SLE.
45
Involvement of both the central (CNS) and peripheral
(PNS) nervous systems occurs. Central nervous system
disorders range from diffuse processes such as headache,
psychosis, and mood disorders to more focal processes
such as seizures. Headaches are reported in more than
50% of persons with SLE, with both migrainous and
tension-type headaches being described.
45
Psychotic man-
ifestations include depression and unnatural euphoria, as
well as decreased cognitive functioning, and confusion.
Chronic discoid cutaneous lupus
is a disease char-
acterized by plaquelike lesions on the head, scalp, and
neck. These lesions first appear as red, swollen patches
of skin, and later there can be scarring, depigmentation,
and plugging of hair follicles. The disease is usually
confined to the skin, but after many years 5% to 10%
of persons with the disorder may develop multisystem
manifestations.
Subacute cutaneous lupus erythematosus
is a less-
severe form of lupus that presents with skin lesions
resembling psoriasis on sun-exposed areas such as the
face, chest, upper back, and arms. Most persons have
mild systemic manifestations of SLE, which usually are
limited to joint and muscle pains. There is also a low
incidence of lupus nephritis.
Diagnosis and Treatment.
The diagnosis of SLE is
based on a complete history, physical examination, and
CNS symptoms
Baldness
Oral ulcers
Butterfly rash
Pericarditis
Myocarditis
Lupus
glomerulonephritis
Lymphadenopathy
Fingertip lesions
Osteoporosis
Myositis
Polyarthritis
Splenomegaly
Anemia
Neutropenia
Thrombocytopenia
Pleuritis
Pneumonitis
FIGURE 44-7.
Clinical manifestations of systemic lupus
erythematosus. CNS, central nervous system.
