Colchicine monograph. Facts & Comparisons eAnswers. St. Louis, MO: Wolters Kluwer Health; 2010.
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Cronstein BN, Terkeltaub R. The inflammatory process of gout and its treatment. Arthritis Res Ther.
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renal disease. Am J Med. 1982;72:95.
Ninety percent of patients who are affected by systemic sclerosis also
have signs and symptoms consistent with Raynaud phenomenon.
Nifedipine, prazosin, and losartan may decrease severity and frequency
of symptoms of Raynaud syndrome.
Many authorities believe polymyalgia rheumatica and temporal arteritis
are manifestations of the same underlying process occurring at different
times during the clinical course. However, they each have characteristic
symptoms and are treated differently.
Reactive arthritis is secondary to certain genitourinary, gastrointestinal,
or respiratory infections. Patients with the HLA-B27 gene are more
susceptible to spondyloarthritis.
Patients with documented active infections, and their partners when
caused by a sexually transmitted disease, should be offered antibiotics.
However, efficacy of prolonged antibiotics as a treatment of reactive
Nonsteroidal anti-inflammatories are appropriate initial therapy for
reactive arthritis to control pain and inflammation.
In severe or prolonged cases of reactive arthritis, steroids, DMARDs,
and biologicals may be considered.
the disease is not controlled with corticosteroids alone, if the response is
positive but steroid sparing effects are desired, or if the condition
presents initially with extramuscular complications.
Despite new knowledge in the immunology and the pathogenesis of the different
connective tissue diseases (CTDs), the etiology of these conditions remains unclear.
Diagnosing CTDs can be difficult because of the complexity of the diseases and the
varying presentation of symptoms. The patient’s reported history of symptoms, results
of the physical examination, and laboratory testing help guide the diagnosis of a
It has been reported that as many as half of patients who have a diagnosed
CTD had been identified as having undifferentiated connective tissue disease.
take years for a patient to be diagnosed and meet classification criteria. Diffuse
CTDs include systemic lupus erythematosus (SLE), scleroderma, polymyositis,
dermatomyositis, rheumatoid arthritis, and Sjögren syndrome. Patients may present
with findings consistent with more than one CTD, and symptoms generally do not all
appear simultaneously. Mixed CTD is an overlap of autoimmune disease features that
can include myositis, scleroderma, and lupus.
Many patients such as those with SLE can have arthralgias and arthritis as part of the
inflammatory disease associated with their CTD. Inflammatory disease is suggested
by morning stiffness of greater than 1 hour (a similar problem occurs with sitting or
resting), swelling, fever, weakness, and systemic fatigue. In some patients, activities
of daily living and function may be excellent despite pain and deformity; in others,
because of psychologic and systemic disease, there may be poor function with
minimal articular involvement. Other psychosocial aspects of their life, including
sexuality, may be affected by many of the inflammatory disorders.
Dermatologic changes are often associated with a particular rheumatic disease.
Examples include alopecia with SLE, onycholysis and keratoderma blennorrhagica
with reactive arthritis, buccal or genital ulcers with SLE or Reiter syndrome,
Raynaud phenomenon with SLE or systemic sclerosis, calcinosis and rash over the
knuckles (Gottron papule) with dermatomyositis, and sun sensitivity malar rash with
SLE. The presence of nodules, tophi, telangiectasia, or vasculitic changes also may
be detected, helping the clinician differentiate which inflammatory disease is present
and what management is necessary.
The CTDs are commonly associated with musculoskeletal changes. Joints may
display warmth, redness and effusion, synovial thickening, deformities, decreased
range of motion, pain on motion, tenderness on palpation, and decreased function.
Often, a patient’s hand and arm function, as well as gait, may be altered. In addition
to the signs and symptoms used to differentiate various rheumatic diseases,
laboratory evaluation of patients with rheumatic complaints can often define the
extent of disease or detect other organ systems that may be involved.
SELECTED CONNECTIVE TISSUE DISEASES
CTDs and rheumatic diseases encompass a wide range of disorders that are
inflammatory in nature and related to the immune system. The following are some of
the conditions that are encountered in clinical practice and will be discussed in this
chapter: scleroderma, polymyalgia rheumatica, temporal arteritis, reactive arthritis,
polymyositis, and dermatomyositis. The reader is also referred to Chapter 33, which
Systemic Sclerosis (Scleroderma)
Systemic sclerosis, or systemic scleroderma, is a CTD associated with autoimmunity
characterized by excessive extracellular matrix deposition and vascular injury to the
skin and other visceral organs.
3 Systemic sclerosis can be classified into distinct
clinical subsets based on the patterns of skin and internal organ involvement,
autoantibody production, and patient survival.
3 The most common subsets include
limited cutaneous (approximately 60% of patients) and diffuse cutaneous
(approximately 35% of patients).
3 The term overlap syndrome may be applied to
patients when features common in one or more of the other CTDs are present and
affect approximately 11% of patients diagnosed with systemic sclerosis. The limited
cutaneous subset is diagnosed when skin thickening is limited to the areas distal to
the elbows and knees. A constellation of dysfunctions known as CREST (calcinosis
cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia)
syndrome is a variant of limited cutaneous systemic sclerosis.
More women than men suffer from systemic sclerosis (female- to-male ratio,
4.6:1), although the mean age at diagnosis does not differ between men and women.
Onset of systemic sclerosis generally begins in adults between 30 and 50 years of
age and is rare in children and seniors older than 80 years of age.
estimated at 276 cases/million adults in the United States.
patients are twice as likely as non–African-American patients to have diffuse
disease, with the annual incidence in African-American women being twice that of
4,6 Likely and possible risk factors for the disease include
environmental exposure to silica dust (e.g., coal miners) and the presence of a
connective tissue growth factor polymorphism, respectively.
conclusive evidence to support an association between silicone breast implants and
systemic sclerosis, or any other CTD.
7 Cigarette smoking is not associated with an
increased risk of systemic sclerosis.
The underlying pathophysiologic changes that lead to systemic scleroderma remain
unknown, but many believe that it results from a lymphocyte-mediated autoimmune
reaction with endothelial cells, activated immune cells, and fibroblasts playing a key
role in the process. It is hypothesized that the process is initiated by an immune attack
on the endothelium leading to endothelial cell activation or injury. This is followed
by activation of the fibroblasts, resulting in subendothelial connective tissue
proliferation, narrowing of the vascular lumen, and Raynaud phenomenon. T cells are
then selectively activated and populate the affected areas such as the dermis and lung
tissue. These cells produce cytokines that stimulate resident fibroblasts to produce
excessive amounts of procollagen, which is then converted extracellularly to mature
collagen. Later, when the inflammatory process subsides, the fibroblasts revert back
to normal. The most common, and serious, complications of systemic scleroderma
involve the pulmonary system and may include fibrosis or interstitial lung disease as
well as pulmonary vascular disease leading to pulmonary arterial hypertension. It is
estimated that pulmonary involvement occurs in 40% of individuals and contributes
from 13% to 17% of deaths from systemic scleroderma.
include, but are not limited to, poor wound healing, arrhythmia, heart failure, renal
failure, and esophageal strictures.
QUESTION 1: T.P., a 58-year-old African-American woman with known limited cutaneous scleroderma,
T.P. is complaining of classic symptoms of Raynaud syndrome, which is a
common clinical feature of limited cutaneous scleroderma, present in over 95% of
cases. In addition, skin fibrosis that is present distal to the elbow or knees, and
telangiectasias are suggestive of limited cutaneous scleroderma as opposed to diffuse
cutaneous scleroderma. Because ANAs may be present in unaffected patients and
absent in afflicted patients, ANA test results must be interpreted within the clinical
context and should not be relied on as a sole diagnostic marker.
CASE 46-1, QUESTION 2: Based on these signs and symptoms, which variant(s) of limited cutaneous
scleroderma is likely present?
Over 95% of patients who are affected by systemic sclerosis also have signs and
symptoms consistent with Raynaud phenomenon, as it is seen with T.P. Patients will
typically complain of recurrent, intermittent vasospastic episodes resulting in a color
change in the fingers or toes after being exposed to cold temperatures.
Vasoconstriction may lead to local cyanosis and accompanying pain and numbness,
with flushing noted on rewarming. Other parts of the body may also be affected, such
as the nose, ears, tongue, and nipples.
Common Clinical Features of Systemic Sclerosis
Subset Skin Fibrosis Lung Involvement
Limited cutaneous Areas distal to the
Diffuse cutaneous Areas proximal or
GERD, gastroesophageal reflux disease.
disease. Am Fam Physician. 2008;78:961.
Common clinical features can be used to distinguish between limited and diffuse
cutaneous systemic sclerosis subsets (Table 46-1).
condition may exist within each subset based on the presence of other symptoms.
Manifestations of systemic sclerosis differ based on the organ system(s) involved
3 Other disorders that may have similar clinical characteristics, such as
amyloidosis and mixed CTD, should be considered and ruled out before the
diagnosis of systemic sclerosis. The ACR/EULAR criteria for the diagnosis of
systemic sclerosis require the presence of one lone major criterion (skin thickening
of fingers of both hands extending proximal to metacarpophalangeal joints) or two
minor criteria (skin thickening of fingers, fingertip lesions, telangiectasia, abnormal
nailfold capillaries, pulmonary arterial hypertension and/or interstitial lung disease,
Raynaud phenomenon, or presence of any of systemic sclerosis-related
10 Skin biopsy is recommended to confirm scleroderma if the clinical
picture is unclear. Separate criteria exist for the diagnosis of juvenile systemic
10 The overall course of systemic sclerosis is highly variable and
unpredictable. However, after a remission occurs, relapse is uncommon.
Manifestations of Systemic Sclerosis
Cardiovascular Abnormal cardiac conduction, congestive heart failure, pericardial effusion,
digital ischemic changes, Raynaud phenomenon
Gastrointestinal Barrett esophagitis or strictures, gastroesophageal reflux disease, dysphagia,
halitosis, chronic cough, dental erosions
Genitourinary Sexual dysfunction, dyspareunia, impotence
Musculoskeletal Flexion contractures, muscle atrophy, puffy hands, inability to make a tight fist,
Pulmonary Interstitial lung disease, pulmonary arterial hypertension, basilar and course
Skin Calcinosis, pruritus, thickened skin, tight skin, excoriations, scabbing, loss of
disease. Am Fam Physician. 2008;78:961.
CASE 46-1, QUESTION 3: At this time, which therapeutic agent(s) is recommended to treat T.P.’s
manifestations and symptoms of systemic sclerosis?
Clinical practice guidelines exist, which may assist the clinician when determining
treatment for systemic sclerosis.
12 However, there is not a specific therapy for
systemic sclerosis. Rather, treatment is mainly supportive and symptomatic in nature,
targeting the specific organ(s) affected (Table 46-3).
13 Therefore, the primary goals
of therapy are to improve the quality of life and minimize the risk of complications.
Based on T.P.’s current symptoms, initiating therapy with the dihydropyridine
calcium-channel blocker nifedipine is an appropriate option to achieve symptom
control. Compared with placebo, nifedipine and prazosin modestly reduced the
severity and frequency of Raynaud ischemic attacks.
14,15 However, when losartan was
compared with low-dose nifedipine in a nonblinded, randomized, controlled trial
(RCT), losartan users experienced a decrease in severity and frequency of Raynaud
16 This should not be considered as definitive
evidence that losartan is superior as the lack of blinding may have resulted in an
overestimation of losartan’s benefit. Although tadalafil monotherapy does not appear
effective for Raynaud phenomenon due to systemic sclerosis, when added to a
calcium-channel blocker the combination appears to improve symptoms and reduce
digital ulcers compared to calcium-channel blocker alone.
restricted in the United States and approved for the treatment of symptomatic
pulmonary hypertension) has been shown to decrease the occurrence of digital ulcers
19 Compared to placebo, atorvastatin 40 mg daily
reduced the number of new digital ulcers in patients with Raynaud phenomenon and
systemic sclerosis during a four-month randomized trial: mean number of new digital
ulcers per patient 1.6 versus 2.5 comparing atorvastatin versus placebo,
20 Patients who develop pulmonary artery hypertension early the
treatment with an angiotensin-converting enzyme inhibitor may improve prognosis in
12,21 For patients who develop pulmonary hypertension,
treatment with bosentan, ambrisentan, sildenafil, epoprostenol infusion, or other
prostanoids (treprostinil, iloprost) may be considered because they have all been
shown to improve functional capacity.
7 The effects of cyclophosphamide, an
antineoplastic agent, demonstrated in clinical trials are conflicting. In an RCT
comparing cyclophosphamide with placebo in patients with scleroderma lung
disease, use of this agent modestly reduced dyspnea and disability while improving
22 However, a meta-analysis of three RCTs and six cohort studies
concluded that cyclophosphamide does not result in significant clinical improvement
23 Because this is a potentially toxic drug, patients who take it
Treatment Options for Manifestations of Systemic Sclerosis
Manifestation Treatment Options
Raynaud phenomenon Nifedipine, verapamil, losartan, prazosin, iloprost, calcium-channel blocker +
Pulmonary hypertension Bosentan, sildenafil, enalapril, iloprost
Interstitial lung disease Cyclophosphamide, prednisone
Renal crisis Angiotensin-converting enzyme inhibitors, dialysis, or kidney transplant
Skin fibrosis Methotrexate, cyclosporine, D-penicillamine
Arthralgias Acetaminophen and NSAIDs
GERD Proton pump inhibitors, H2 antagonists, prokinetic agents
Pruritus Antihistamines, low-dose topicalsteroids
al. Database Online. October 15, 2009. Hoboken, NJ:John Wiley & Sons. Accessed March 18, 2011.
Polymyalgia Rheumatica and Temporal Arteritis (Giant
Polymyalgia rheumatica (PMR) and temporal arteritis, also known as giant cell
arteritis (GCA), are closely related clinical syndromes that usually affect the elderly
and frequently occur together. Many authorities believe them to be different phases of
the same underlying disease process. Polymyalgia rheumatica is 2 to 3 times more
common than GCA. However, about 27% to 53% of persons with GCA also have
PMR, and 18% to 26% of those with PMR also have GCA.
with, or after the development of GCA. Inflammation is the hallmark of both
conditions. PMR is characterized by aching and morning stiffness in the cervical
region and shoulder and pelvic girdles, and may result in disability.
as a result of GCA most commonly involves the temporal artery, but arteries in other
parts of the body can also be affected.
26 Despite certain similarities, PMR and GCA
have distinct symptoms, corticosteroid requirements for treatment, and prognoses.
The incidence of PMR and GCA increases in patients after the age of 50 and peaks
in those 70 to 80 years of age.
25 GCA is the most common vasculitis among the
elderly and can lead to blindness if not diagnosed and treated in a timely manner.
Likewise, without treatment, PMR can lead to significant morbidity and disability.
Patients with PMR are also at an increased risk of peripheral arterial disease. The
primary risk factor for both conditions is age, and both occur more frequently in
women than in men. PMR is seen mainly in people of north European ancestry and
generally affects whites more commonly than African-Americans, Hispanics, Asians,
26 The incidence of PMR is 5.9/10,000 patients/year in the
United States, with an overall prevalence in the United States of about 740/100,000:
532 for men and 925 for women.
27 The incidence of GCA is 0.17 new cases annually
per 1,000 persons older than 50 years of age with a prevalence of 2/1,000 persons.
Although the exact pathogeneses of PMR and GCA are yet to be determined, they
are both thought to arise from an autoimmune or inflammatory dysfunction involving
similar cellular immune responses from T cells, antigen-presenting cells,
macrophage-derived inflammatory cytokines, genetic human leukocyte antigen
molecules, and macrophages. A viral cause has been suspected but not confirmed for
both PMR and GCA, and some studies demonstrate a cyclic pattern in PMR
incidence pointing to environmental infectious triggers (e.g., parvovirus B19,
Mycoplasma pneumoniae, and Chlamydia pneumoniae) as potential causes.
Branches of the internal and external carotid arteries are most commonly affected in
those with GCA, and biopsies often reveal inflammatory changes that lead to a
narrowing or occlusion of the vessel and ischemia distal to the lesion.
inflammation is the most prominent feature in PMR, but inflammation of the blood
vessels is often clinically undetectable.
QUESTION 1: D.C. is an 85-year-old white man presenting to the emergency department complaining of
new-onset morning aching pain and stiffness in his shoulders and upper arms. He states his symptoms
No conclusive laboratory test for PMR or GCA exists, and nonspecific clinical
features and the absence of physical signs often complicate diagnosis. Appropriate
diagnosis requires a thorough history and physical examination. Distinguishing
between the two disorders is of importance, as GCA can lead to blindness and
requires higher doses of treatment medications. The typical onset of PMR is acute in
nature. However, as is the case with D.C., most who present for a medical evaluation
describe their symptoms as occurring for 1 month or longer.
common complaints associated with PMR, which include aching pain and morning
stiffness in the shoulders and upper arms, hips and thighs, or neck and torso. The
shoulders are affected in 95% of cases. New-onset GCA often manifests as a new
headache or a headache that is described as different from previous headaches and
has been occurring for 2 to 3 months. Constitutional symptoms such as fatigue,
anorexia, and weight loss in an older patient often accompany headaches seen in
24 The absence of headache in D.C. further supports the diagnosis of PMR.
Common findings associated with both conditions are presented in Table 46-4.
most useful laboratory test for diagnosing PMR is the ESR. Patients with GCA
usually have an ESR greater than 40 to 50 mm/hour; rates of greater than 100
mm/hour are common. A normal ESR is very helpful in ruling out GCA in
corticosteroid-naïve patients; however, an elevated ESR (>100 mm/hour) is only
minimally helpful in ruling out GCA.
30 Three or more of the following criteria that
are present are 93% sensitive and 91% specific for GCA: age of onset of disease
greater than or equal to 50 years, new headache, temporal artery abnormality, ESR
greater than or equal to 50 mm/hour, or abnormal findings on biopsy of the temporal
31 The BSR/BHPR guidelines recommend biopsy of the temporal artery for the
32 An abnormal biopsy in the context of positive clinical findings
diagnosis of GCA if there is strong clinical suspicion.
Common Findings Associated with Polymyalgia Rheumatica and Giant Cell
Polymyalgia Rheumatica Giant Cell Arteritis
ESR >50 mm/hour ESR >50 mm/hour
Anemia (mild, normochromic, normocytic) Anemia
Aching, pain, and morning stiffness in the shoulders and
upper arms, hips and thighs, or neck and torso
Headache: temporal with temporal artery involvement,
or occipital with occipital artery involvement
Symptoms of systemic inflammation Visualsymptoms or jaw claudication
Fever, weight loss, depression, fatigue
ESR, erythrocyte sedimentation rate.
CASE 46-2, QUESTION 2: What other inflammatory conditions should be considered and excluded before
making the diagnosis of PMR or GCA in D.C.?
Other inflammatory or autoimmune disorders, such as fibromyalgia, myalgias from
statin therapy, osteoarthritis, polymyositis, and rheumatoid arthritis, should be
considered and excluded before the diagnosis of PMR or GCA.
alternatives that should be included in the differential diagnosis of PMR include
hyperparathyroidism, Parkinson disease, thyroid disorders, adhesive capsulitis,
pseudogout, cervical spondylosis, SLE, or multiple myeloma.
(Wegener granulomatosis, polyarteritis nodosa, microscopic polyangiitis), Takayasu
arteritis, malignancies, herpes zoster, and migraines (or other causes of headaches)
should be included in the differential diagnosis of GCA.
CASE 46-2, QUESTION 3: What is the preferred initial treatment approach for PMR in D.C.? Explain the
difference(s) in this approach as compared with those for GCA.
Owing to its anti-inflammatory properties, corticosteroids are considered first-line
therapy for either PMR or GCA. Early visual loss may occur in up to 20% of patients
and rarely improves once present. Therefore, initiation of corticosteroid treatment
should not be delayed pending temporal artery biopsy results.
occur simultaneously, higher corticosteroid doses (indicated for the treatment of
CGA) are required to prevent significant complications. For the treatment of PMR,
the British Society for Rheumatology (BSR) and the British Health Professionals in
Rheumatology (BHPR) recommend standard initial treatment with oral prednisone 15
mg/day for 3 weeks, 12.5 mg/day for 3 weeks, and 10 mg/day for 4 to 6 weeks, then
decrease by 1 mg every 4 to 8 weeks.
29 An alternative treatment regimen includes
intramuscular methylprednisolone 120 mg every 3 to 4 weeks, decreased by 20 mg
every 2 to 3 months. Beginning low-dose prednisone may improve D.C.’s PMR
symptoms within days, but some optimal results could take several weeks to achieve
and most patients can expect to receive therapy for 1 to 2 years. Treatment should be
tailored to patient symptoms, and inflammatory markers should be followed.
Attempts to taper the dose to avoid long-term adverse drug events (e.g., osteoporosis,
hypothalamus–pituitary–adrenal axis suppression) should be made once acute relief
has been achieved. Tapering the dose (e.g., 1 mg/day/week) should be individualized
and based on symptom response because it may take years owing to symptom flares.
The Polymyalgia Rheumatica Activity Scale, a disease activity assessment, may be
used to monitor and adjust therapy and patient response.
As opposed to PMR, treatment for uncomplicated (no jaw or tongue claudication
or visual changes) GCA should begin with high-dose prednisolone (40–60 mg/day).
Complicated GCA (evolving visual loss or history of amaurosis fugax) should be
treated with IV, methylprednisolone 500 mg to 1 g/day for 3 days, followed by oral
29 A corticosteroid taper can be initiated after 4 weeks of
treatment and resolution of symptoms and normalization of ESR/CRP. It is
recommended to decrease the prednisolone dose by 10 mg every 2 weeks until 20 mg
is reached, then decrease by 2.5 mg every 2 to 4 weeks until 10 mg is reached, and
then decrease by 1 mg every 1 to 2 months.
29 Most will achieve a dose of 7.5 to 10
mg/day after 6 months of therapy, but relapses are common and are managed by
restarting steroid therapy or increasing the dose to previous beneficial amounts.
use of low-dose aspirin may reduce the incidence of cranial ischemic complications.
The use of methotrexate as adjuvant therapy for PMR and GCA is not routinely
recommended, and its evidence of symptom relief benefit is conflicting.
single RCT demonstrated that the use of methotrexate 10 mg once weekly in addition
to prednisone in those with GCA resulted in an overall decrease in the amount of
prednisone required and in relapse rates.
35 Relapses are common in both PMR and
GCA and may be managed by restarting steroid therapy or increasing the dose to
previous levels that controlled symptoms, if already receiving steroids. All patients
on long-term corticosteroids should be offered calcium (1,200 mg/day) and vitamin
D (800 international units/day) for prevention of osteoporosis and be monitored for
other complications of steroid therapy. Patients at risk for gastritis should receive
prophylactic protection with a proton pump inhibitor.
Patients being treated for PMR or GCA should be monitored closely in response to
treatment and disease progression. It is recommended that the first follow-up visit
should take place 1 to 3 weeks after the start of corticosteroids.
should occur at 6 weeks as well as 3, 6, 9, and 12 months, or on an as-needed basis
if relapses or adverse events occur. At each visit, patients should be assessed for
specific symptom improvement and routine blood work should include ESR, CRP,
CBC, and CMP. Bone mineral density testing every 2 years may be considered in
patients receiving long-term corticosteroids. Professional guidelines recommend a
chest X-ray every 2 years to assess for aortic aneurysm in patients diagnosed with
29 However, a systematic review of imaging studies found limited evidence to
guide screening recommendations for thoracic aortic aneurysm/dilation because a
chest X-ray may not be sensitive enough to detect thoracic aortic aneurysms.
Reactive arthritis is defined as peripheral arthritis often accompanied by one or more
extra-articular manifestations that appear after certain infections of the genitourinary,
gastrointestinal, or respiratory infection. Table 46-5 lists bacterial pathogens
associated with the development of reactive arthritis.
features with other forms of spondyloarthropathies, which is the grouping it falls
under. Reiter syndrome was previously used to describe this condition, but the term
reactive arthritis has become the preferred term. The term classic “Reiter triad”
associated with this condition was used to describe the specific combination of
arthritis, urethritis, and conjunctivitis.
37 The presentation of arthritis is usually a large
joint monoarthritis or oligoarthritis usually in the lower extremities, and/or enthesitis
which is the inflammation of the entheses. This is the site where the tendon or
36 Typically, the inflammatory disorder would occur 1 to 6
37 The clinical course of reactive arthritis is variable and
usually runs a self-limited course of 3 to 12 months.
arthritis is not common and results from cardiac complications such as aortitis.
Approximately 10% to 20% of patients may continue to have chronic, destructive,
and disabling arthritis or enthesitis within 2 years after the onset of symptoms. Ten to
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