Connective Tissue Disorders and Autoimmune Conditions
Caplan syndrome (i.e., RA
in the setting of coal worker’s pneumoconiosis).
■ Pleural and pericardial disease: Pleural thickening and effusions are the most common thoracic manifestations of RA
with a prevalence of up to 70%, but imaging findings are nonspecific ( Fig. 59.8 ).
■ Osseous changes: Symmetric erosions in the distal clavicles and humeral heads may provide a helpful clue to the diagnosis
of pulmonary involvement by RA
( Fig. 59.9 ).
■ Large airway involvement: There are cricoarytenoid abnormalities in up to 75% of patients with RA
due to the presence
Raashid Luqmani, Maarten Boers, and Theodore Pincus (eds)
lymphadenopathy. Often it is impossible to distinguish polymyalgia rheumatica from polymyalgic onset RA
– some authors have suggested that PMR is a form of rheumatoid factor-negative RA
. In practice, it is usual to institute treatment for PMR (typically 10–15 mg per day prednisolone.) If, despite improvement in their systemic symptoms, the patients develop small joint pain and swelling, this suggests that the diagnosis
was actually “polymyalgic–onset” RA
. A diagnosis
is reinforced by finding that the patient has a positive rheumatoid factor test, or ACPA, if available
Kerry Wright and Lynne S. Peterson
microbiome is likely another source for citrullination.
, a breakdown occurs in the person’s tolerance toward these citrullinated proteins, leading to production of anti–citrullinated protein antibodies (ACPAs). The antibodies are distinct to RA
and can occur 3 to 7 years before the RA diagnosis
. Epitope spreading (the development of antibodies against similar molecules) and increased cytokine production gradually increase the person’s autoimmunity until RA
disease becomes apparent.
Microbial or mechanical insults have been proposed as damaging
Joel David, Anne Miller, Anushka Soni, and Lyn Williamson
1. Give a differential diagnosis
at her initial presentation with joint pains.
2. What are the current recommendations for management of rheumatoid arthritis ( RA
3. What are the causes of shortness of breath in RA
? Give a differential diagnosis
based on the CXR findings.
4. What is the differential diagnosis
for the endomyocardial inflammation or fibrosis?
5. What are the current NICE guidelines for starting biological therapy in RA
The patient had a coronary angiogram which was normal, excluding an ischaemic
Dafna D. Gladman, Cheryl F. Rosen, and Vinod Chandran
( Figure 7.1 ). If a patient with polyarthritis and psoriasis has a rheumatoid nodule, it is most likely that they have RA
and psoriasis. Rheumatoid nodules should not occur in patients with PsA. On the other hand, if a patient has evidence of dactylitis, or enthesitis, they are more likely to have PsA than RA
. The presence of axial disease, based on inflammatory back or neck pain and stiffness and radiographic evidence of sacroiliitis or syndesmophytes, again points to the diagnosis
of PsA rather than RA
. A clinical feature which helps differentiate RA
Derrick J. Todd and Jonathan S. Coblyn
patients with RA
No single laboratory test should be used to diagnose RA
in the absence of clinical findings. Laboratory studies are used to support the diagnosis
in a patient with suggestive symptoms, to monitor RA
disease activity, and to rule out other possible causes of arthritis.
Serum rheumatoid factor (RF) testing is highly useful in the diagnostic workup of patients with polyarthritis, as approximately 80% of patients with RA
have RF detectable in the serum (termed “seropositive”). The remaining 20% of RA
Postanesthesia Care Unit
Perin Kothari and Sree Kolli
joints that are involved may have stiffness and may have tenderness and warmth to the touch.
What is the primary treatment of RA
, and what are your concerns with these medications?
Once the diagnosis
is made clinically and with laboratory studies, patients are started on medications that can aid in preserving function in those affected joints. These medications are known as disease-modifying antirheumatic drugs. RA
patients often are taking pain medications ranging from nonsteroidal anti-inflammatories to chronic opioid therapy for management of their
Genomics in Clinical Practice
Kate McAllister and Stephen Eyre
to patients who have clinical synovitis in one joint that is not better explained by any other diagnosis
. Once this has been determined, patients who then achieve a point total of 6/10 or higher across four different areas of diagnosis
are classified with “definite RA
.” The four areas of diagnosis
include joint involvement, serological parameters (including anti-CCP and RF), markers of inflammation (including ESR and CRP), and symptom duration. Differentiating early RA
from self-limiting or other forms of inflammatory arthritis is challenging. Radiographic changes
Manoj Sivan, Margaret Phillips, Ian Baguley, and Melissa Nott
shown to be associated with development of RA
is arrived at through a combination of clinical and laboratory tests. Onset is insidious with inflammation, pain, and stiffness present in synovial joints. The small joints (metacarpophalangeal, proximal interphalangeal, wrist, and metatarsophalangeal joints) are commonly affected with symmetrical changes ( Fig. 35.2 ). Laboratory tests and radiographs are used to support the clinical impression of RA
and exclude other differential diagnosis
. The American College of Rheumatology and
Anthony B. Ward, Michael P. Barnes, Sandra C. Stark, and Sarah Ryan
Laboratory tests results in a patient with active RA
• Raised inflammatory markers—C-reative protein (CRP) and erythrocyte sedimentation rate (ESR). • Positive immunoglobulin M—rheumatoid factor occurs in 75% of RA
patients. It is possible to have RA
with a negative rheumatoid factor, where the diagnosis
is classified as sero-negative RA
. • Acute phase response including raised gamma GT, alkaline phosphatase and ferritin and a reduction in serum albumin and haemoglobin. Radiological changes that can occur with RA
• Joint erosions. • Periarticular osteoporosis.
John R. Schairer and Steven J. Keteyian
demonstrating RV collapse.
Increased pericardial pressure also causes RA
collapse or inversion ( Fig. 166.2 ) [ 11 ]. The percentage of the cardiac cycle that RA
inversion occupies is termed the RA
inversion time index. An index ≥0.34 yielded 94% sensitivity, 100% specificity, predictive value of 100%, and accuracy of 97%. The index is believed to be the most sensitive finding for tamponade. The absence of sinus rhythm does not preclude the use of RA
collapse to make the diagnosis
of tamponade. Increased intrapericardial pressure also explains the loss of the
Joel David, Anne Miller, Anushka Soni, and Lyn Williamson
A 55-year-old woman with a history of seropositive erosive RA
for 7 years attended the ‘flare’ clinic with a 1-day history of an acutely swollen, red, and painful right wrist, associated with increased fatigue and fever. She had been on weekly etanercept 50 mg for the previous year. In clinic 2 weeks previously, her joints were not inflamed and the DAS28 was 3.0.
Her medical history included a recent diagnosis
of hypertension, for which bendroflumethiazide had been prescribed.
In clinic her temperature was 37.5°C. Examination of the right wrist
Richard Watts, Gavin Clunie, Frances Hall, and Tarnya Marshall (eds)
membranous glomerulonephritis (GN) is recognized. More common renal lesions in an RA
patient might be GN or tubulointerstitial nephritis from the toxic effects of drugs. Investigations No pathognomonic test result exists for the diagnosis
; therefore, the investigation of polyarthritis is driven by clinical suspicion. Laboratory • FBC shows associated anaemia (either normochromic normocytic or mildly hypochromic microcytic) and thrombocytosis. • Neutropaenia may point to a diagnosis
of Felty's syndrome though isolated mild neutropaenia is not an uncommon finding
Stephen Chapman, Grace Robinson, John Stradling, Sophie West, and John Wrightson
and standard tests Differential diagnosis
and standard tests
Rheumatoid arthritis ( RA
) Rheumatoid arthritis ( RA
Systemic lupus erythematosus (SLE) Systemic lupus erythematosus (SLE)
Polymyositis and dermatomyositis Polymyositis and dermatomyositis
Systemic sclerosis Systemic sclerosis
Sjögren’s syndrome Sjögren’s syndrome
Ankylosing spondylitis Ankylosing spondylitis
Behçet’s syndrome Behçet’s syndrome
Autoantibodies: disease associations Autoantibodies: disease associations
Susan M. Oliver (eds)
Seropositivity of the RA
• Absence of RF does not preclude diagnosis
but is a prognostic indicator of aggressive disease with a poorer outcome. Anti-CCP serology has been shown to have a higher specificity and sensitivity than RF.
• Social and psychological factors that may reflect poor outcomes ( see ‘ Education Education, social , and Social and psychological aspects of a new diagnosis
psychological issues ’ , pp. Education 336 – Social and psychological aspects of a new diagnosis
Rheumatoid arthritis: assessment
is a long-term
Clinical presentations of rheumatic disease in different age groups
pattern which may mimic RA
in addition to an oligoarthritis or axial disease. Nail dystrophy is usual and psoriatic plaques on the elbows may indicate the diagnosis
. For a review of the clinical features see Cantini et al. 22
Nodal ostoarthritis is one of several clinical presentations of osteoarthritis, typically occurring after 40 years of age in women and affecting the DIPs, PIPs, and thumb base although the MCPs are not infrequently affected. Inflammatory forms may mimic RA
or psoriatic arthritis. For a recent consensus report on diagnosis
see Zhang et al. 23
Rebecca Jester, Julie Santy, and Jean Rogers
knees or shoulders. The symptoms may be confined to these areas or become more generalized affecting other joints • RA
has a rapid or insidious onset • The presence of inflammation in the joints (especially the small joints of the hands and feet) should prompt early referral for diagnosis
Common symptoms patients with active RA
can experience include: • Joint pain • Joint swelling • Stiffness (often more marked on waking) • Fatigue • Low mood. RA
has extra-articular and systemic manifestations including:
• Anaemia • Weight loss • Vasculitis • Rheumatoid lung
Kapil Sugand, Miriam Berry, Imran Yusuf, Aisha Janjua, Chris Bird, David Metcalfe, Harveer Dev, and Sri Thrumurthy (eds)
are there any other signs of SSc or SLE? Does the patient with apparently rheumatoid hands have any nail dystrophy or psoriasis that might alter your differential diagnosis
? Most students are easily able to recognize RA
, but often struggle to describe it; if you can produce a smooth, sequential presentation supporting your diagnosis
, you will please the examiner.
Clinical signs of RA
(See Fig. 31.2 .) The most likely case is a rheumatoid hand. Students are very good at identifying a rheumatoid hand (severe cases are obvious) but often struggle
Susan M. Oliver (eds)
management. There is a differential diagnosis
, most commonly crystal arthropathy. Unless a non-infective diagnosis
can be made with confidence, an emergency referral to 2° care should be made. If diagnostic doubt remains, treat for septic arthritis until proven otherwise.
Box 13.1 Septic arthritis: overview
Risk factors for septic arthritis (examples)
• Pre-existing joint disease, particularly RA
, joint replacement.
• Older age, diabetes mellitus, chronic renal failure, or alcoholism.
• Immunosuppression (e.g. AIDs, hypogammaglobinaemia).
Ian McNab and Chris Little
affected by rheumatoid synovitis, often with radiographic changes, but because symptoms are similar to those of rotator cuff disease it is important to determine the source of pain. Box Box 10.1 Typical features of ACJ involvement in RA
10.1 shows the typical features of ACJ involvement. Box 10.1 Typical features of ACJ involvement in RA
• Pain well localized to the joint • Pain reproduced by high arc (>120 degrees – end of range) elevation movements (abducting the outstretched arm above the patients head) and by horizontal flexion (crossing the arm towards the opposite