Extra-articular features associated with inflammatory rheumatological diseases

Inflammatory rheumatological diseases may present with nonspecific systemic symptoms, including prodromal symptoms, fatigue, night sweats, weight loss and myalgia. They can also present with extra-articular features that help to define specific syndromes.

Extra-articular features of specific rheumatological diseases include (this list is not exhaustive):

rash and nail changes (eg psoriatic plaques in psoriatic arthritis, rash in dermatomyositis)
eye inflammation (eg uveitis in spondyloarthritis and scleritis in inflammatory arthritis, such as rheumatoid arthritis)
respiratory features (eg interstitial lung disease in some systemic vasculitides and rheumatoid arthritis)
neurological features (eg long-tract neurological signs in myelopathies)
gastrointestinal features (eg dysphagia in systemic sclerosis)
cardiovascular features (eg pericarditis in systemic lupus erythematosus, valvular disease in antiphospholipid syndrome)
genitourinary features (eg glomerulonephritis in some systemic vasculitides).

Clinical features suggestive of serious rheumatological pathology requiring urgent management (also known as ‘red flags’) are outlined in Serious rheumatological conditions requiring urgent management and their alerting features (‘red flags’) in adults. These features are considered by the Rheumatology Expert Group to suggest potentially serious, organ- or life-threatening rheumatological disease. Refer people with ‘red flag’ features for urgent assessment by an experienced clinician. Multiple presentations to an emergency department with the same symptoms should also raise suspicion for the presence of serious pathology.

Note: Seek urgent specialist advice if any of the ‘red flag’ features are present.

Table 1. Serious rheumatological conditions requiring urgent management and their alerting features (‘red flags’) in adults
[NB1]
Serious rheumatological condition	Alerting features (‘red flags’)
septic arthritis	acute unexpected monoarthritis (rarely oligo- or polyarthritis) systemically unwell (eg fever, tachycardia, hypotension [rare]) raised inflammatory markers (serum CRP concentration, ESR) history of immunosuppression or currently taking immunomodulatory drugs history of intravenous drug use
severe or sight-threatening giant cell arteritis	older person (usually over 70 years) new-onset headache acute visual disturbance (eg diplopia, amaurosis fugax [NB2], visual field defect) jaw claudication [NB3]—almost pathognomonic for giant cell arteritis
sight-threatening acute uveitis / scleritis	acute unilateral (usually), painful, photophobic, watering red eye uveitis—potentially associated with spondyloarthritis scleritis—potentially associated with rheumatoid arthritis or other autoimmune diseases (eg granulomatosis with polyangiitis, Crohn disease, ulcerative colitis)
acute myelopathy (spinal cord injury)	acute back or neck pain associated with long-tract neurological signs
acute cauda equina syndrome	acute back pain associated with altered bladder and/or bowel function and reduced perianal sensation
organ-threatening ischaemia associated with systemic vasculitis	signs of acute organ impairment affecting a major organ or body system (eg glomerulonephritis, pulmonary haemorrhage, intestinal ischaemia, sight-threatening eye disease, neuropathy, stroke)
Note: CRP = C-reactive protein; ESR = erythrocyte sedimentation rate NB1: This list is not exhaustive. NB2: Amaurosis fugax is an acute, temporary, unilateral, painless visual loss associated with retinal ischaemia. It is commonly associated with giant cell arteritis. NB3: Jaw claudication is the sensation of jaw pain on mastication, which settles at rest. It is commonly associated with giant cell arteritis.