MED: Rheumatology Flashcards
What is the management of fibromyalgia?
- explanation
- aerobic exercise: has the strongest evidence base
- cognitive behavioural therapy
- medication: pregabalin, duloxetine, amitriptyline
What is gout?
A rapid onset crystal-induced arthritis
What are RFs for gout?
- Male
- Woman’s risk increases following the menopause
- Increasing age.
- Diet high in purines - meat, seafood and fructose-containing foods.
- Alcohol
- Metabolic syndrome
- Medications - Loop and thiazide diuretics, Low dose aspirin, Levodopa
- Chronic kidney disease
What are the clinical features of gout?
- Most first presentations involve the first MTP
- May affect other peripheral joints - other joints of the feet, ankle, knee, hand, wrist and elbow.
- Rarely affects more central joints such as hip and spine.
Typical features of affected joint:
- Intense pain - stabbing, contact with joint is very painful, may prevent patient from sleeping.
- Erythema - red and warm to touch, may often resemble cellulitis.
- Joint swelling and tenderness resulting in reduced ROM
Systemic symptoms:
- Tophi - in chronic gout, MSU crystals may deposit to form small hard lumps in the tissues surrounding the joint, cartilage and in the joints themselves.
- They may form elsewhere including feet, knees, wrists, ears, fingers, kidneys and sclerae.
- Eye involvement - MSU crystals deposited in cornea (very rare)
What is the crystal type in gout?
Monosodium urate
What is the crystal type in pseudogout?
Calcium pyrophosphate
What are the investigations for gout?
Clinical diagnosis:
- If features suggestive of gout and no suspicions of other conditions e.g. SA
- Supportive features - Mono-articular involvement of a foot/ankle joint, Previous episodes of a similar nature, Rapid onset, Erythema, Male
- If making a clinical diagnosis, screen for cardiovascular risk and risk of kidney disease.
Synovial fluid analysis:
- Indicated if - Diagnosis of gout is unsure, SA is suspected.
- Fine-needle aspiration of the affected joint is the gold standard as the presence of MSU crystals is 100% specific
Serum uric acid:
- Hyperuricaemia is NOT diagnostic of gout but, increased levels correlate with increased risk of developing gout.
- Should be measured 4-6 weeks following acute attack to confirm hyperuricemia.
- Considered raised when >360µmol/L
- Absence of hyperuricaemia does not exclude gout.
Other bloods to consider:
- U&Es: renal function should be measured to ensure appropriate dose of allopurinol.
- FBC: WBC may be raised
- Fasting glucose and lipid profile: gout is associated with metabolic syndrome.
Radiology - useful in chronic gout:
- X-rays usually normal unless the disease is advanced stage.
- In chronic gout, joint effusion is usually the earliest sign. Later, x-rays show punched out lytic lesions, sclerotic margins and outlines of tophi.
- USS: tophi present in chronic gout can be observed via ultrasound appearing hyperechoic (white appearance).
Describe the acute management of gout
NSAIDs or colchicine are first-line:
- Maximum dose NSAID should be prescribed until 1-2 days after symptoms have settled
- Gastroprotection (e.g. PPI) may also be indicated
- Colchicine may be used with caution in renal impairment - BNF advises to reduce dose if eGFR is 10-50 ml/min and to avoid if eGFR < 10 ml/min
- Main side-effect of colchicine is diarrhoea
Second line:
- Oral steroids may be considered if NSAIDs and colchicine are contraindicated.
- Prednisolone 15mg/day is usually used
- Another option is intra-articular steroid injection
If patient already taking allopurinol it should be continued
Describe the long term control of gout
Urate-lowering therapy:
‘Commencement is best delayed until inflammation has settled as ULT is better discussed when the patient is not in pain’
- Allopurinol is first-line
- Dose titrated every few weeks to aim for a serum uric acid of < 360 µmol/l CKS
- A lower target uric acid level below 300 µmol/L may be considered for patients who have tophi, chronic gouty arthritis or continue to have ongoing frequent flares despite having a uric acid below 360 µmol/L
- Colchicine cover should be considered when starting allopurinol. NSAIDs can be used if colchicine cannot be tolerated. May need to be continued for 6 months
- Second-line when allopurinol not tolerated or ineffective = febuxostat
-In refractory cases other agents may be tried - uricase, pegloticase
Lifestyle modifications:
- Reduce alcohol intake and avoid during an acute attack
- Lose weight if obese
- Avoid food high in purines e.g. Liver, kidneys, seafood, oily fish (mackerel, sardines) and yeast products
Other points:
- Consideration should be given to stopping precipitating drugs (such as thiazides)
- Losartan has a specific uricosuric action and may be particularly suitable for the many patients who have coexistent hypertension
- Increased vitamin C intake (either supplements or through normal diet) may also decrease serum uric acid levels
What are RFs for osteoporosis?
- Age (between 40 and 90 years)
- Gender
- Previous fracture
- Parent fractured hip
- Smoking
- Glucocorticoids (more than 3 months at a dose of prednisolone 5mg daily)
- Rheumatoid arthritis
- Secondary osteoporosis
- Alcohol consumption
- BMD
What are the most common pathological fractures?
Vertebral compression fractures:
- Often a sudden episode of acute back pain occurring at rest, bending or lifting
- Restricted spinal flexion and intensified pain with prolonged standing
- Anterior compression fractures in the thoracic spine may lead to thoracic kyphosis (Dowager’s hump)
- Paravertebral muscle spasm and tender upon deep palpation
Appendicular fractures: (fracture of the proximal femur or distal radius following a fall)
- Neck of femur fractures present as hip pain, inability to bear weight and upon physical examination there is a shortened and externally rotated leg
- Colles fractures present after a fall on an outstretched arm, wrist pain and reduced ROM
What are the investigations for osteoporosis?
DEXA scan:
T score - based on bone mass of young reference population
- > -1.0 = normal
- -1.0 to -2.5 = osteopaenia
- < -2.5 = osteoporosis
Plain radiographs:
- Highlight any fractures and reveal previously asymptomatic vertebral deformities.
Investigations to exclude any secondary causes of osteoporosis include:
- Quantitative CT and US of the heel
- History and physical examination
- FBC
- U&Es (serum calcium, creatinine, phosphate)
- LFTs (ALP, transaminases)
- TFTs
- 25-OH vit D & 1,25-OH vit D
- Serum testosterone & prolactin
- Lateral radiographs of lumbar and thoracic spine
- Protein immunoelectrophoresis and urinary Bence-Jones protein
What can be used to evaluate fracture risk of patients?
the FRAX tool
What Clinical risk factors are used in the FRAX tool?
- Age (between 40 and 90 years)
- Gender
- Previous fracture
- Parent fractured hip
- Smoking
- Glucocorticoids (more than 3 months at a dose of prednisolone 5mg daily)
- Rheumatoid arthritis
- Secondary osteoporosis
- Alcohol consumption
- BMD
What is the management of osteoporosis?
Lifestyle modification:
- Falls risk assessment
- Weight-bearing and muscle strengthening exercises
- Optimum daily calcium (800-1200mg) and vitamin D (400-800 IU) intake through sunlight and diet or supplements
- Calculation of 10-year probability of osteoporotic fragility fracture
Oral bisphosphonates:
- First line medication to treat osteoporosis
- Oral Alendronate and risedronate are given as 1-weekly doses, whilst zoledronic acid as a 1-yearly infusion
- They should be taken fasting, with water whilst standing or sitting upright for 30 minutes
- May be associated with upper-GI side effects such as oesophagitis
- Careful monitoring is required in those with CKD4 or 5
Denosumab:
- Monoclonal antibody
- Only used in extensive osteoporosis
- Given as a SC injection every 6 months
- Adverse effects are infrequent but serious - Dysuria, Cellulitis, Osteonecrosis of the jaw
What is the management of a new vertebral fracture?
Bed rest for 1-2 weeks
Strong analgesia
Muscle relaxants (i.e. diazepam 2mg TDS)
Gradual physiotherapy
What is Paget’s disease of the bone?
A disease of increased but uncontrolled bone turnover
What are RFs for Paget’s ?
increasing age
male sex
northern latitude
family history
What bones are most commonly affected in Paget’s?
Skull
Spine/pelvis
Long bones of the lower extremities
What are the clinical features of Paget’s ?
> > Only 5% of patients are symptomatic
- Stereotypical presentation is older male with bone pain and an isolated raised ALP
- Bone pain (e.g. pelvis, lumbar spine, femur)
- Classical, untreated features - bowing of tibia, bossing of skull
What are the investigations for Paget’s?
- Raised ALP
- Calcium and phosphate are typically normal
- Skull x-ray - thickened vault, osteoporosis circumscripta
What is the management of Paget’s?
Indications for treatment include bone pain, skull or long bone deformity, fracture, periarticular Paget’s
- Bisphosphonate (either oral risedronate or IV zoledronate)
- Calcitonin is less commonly used now
What are the complications of Paget’s?
- deafness (cranial nerve entrapment)
- bone sarcoma (1% if affected for > 10 years)
- fractures
- skull thickening
- high-output cardiac failure
What is Polyarteritis nodosa (PAN)?
A vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation
What are the clinical features of PAN?
- fever, malaise, arthralgia
- weight loss
- hypertension
- mononeuritis multiplex, sensorimotor polyneuropathy
- testicular pain
- livedo reticularis
- haematuria, renal failure
- p-ANCA are found in around 20% of patients with ‘classic’ PAN
- hepatitis B serology positive in 30% of patients
What are the investigations for PAN?
Laboratory testing:
Basic laboratory tests help ascertain the extent of involvement of different organs:
- LFTs
- Hepatitis B and C serologies
- Creatinine kinase
- Serum creatinine
- Urine analysis
- ESR and CRP are significantly elevated
Chest radiography:
- May be obtained to exclude other forms of vasculitis, which have greater involvement in the lungs.
Biopsy:
- Performed on a clinically affected organ to confirm the diagnosis.
Imaging:
- Arteriography (mesenteric or renal) can be used as an alternative to biopsy to confirm the diagnosis (to minimise bleeding risk). It can reveal aneurysms and irregular constrictions in the vessels.
- CT/MRI can demonstrate wedge shaped renal infarctions, which are less specific than microaneurysms showed on arteriography.
What is the management of PAD?
Mild disease with constitutional symptoms but no end-organ damage:
- Oral prednisone (1mg/kg body weight).
Mild disease but resistant to or intolerant to of required dose of glucocorticoids:
- Addition of azathioprine or methotrexate to a tolerable dose of glucocorticoids.
- Methotrexate should be avoided in patients with renal disease or hepatitis.
Moderate to severe disease, with evidence of end-organ damage:
- High-dose glucocorticoids and cyclophosphamide, followed by azathioprine or methotrexate for remission maintenance.
- In patients with mild PAN and associated hepatitis B or C infection, it is advised to treat initially with antivirals only, and not to give any immunosuppressants.
- However, patients with severe hepatitis virus-associated PAN may be benefited from short term glucocorticoids or plasma exchange until antiviral therapy becomes effective.
What is Polymyalgia rheumatica?
An inflammatory condition that causes pain and stiffness in the shoulder and pelvic girdles
What are the clinical features of PMR?
Subacute onset (2-6 week history) of:
- Shoulder and/or pelvic girdle pain and stiffness lasting at least 45 minutes (may be initially unilateral but then becomes bilateral).
- Shoulder girdle pain - can be accompanied by tenderness, signs of bursitis and restriction of shoulder movement (e.g. difficulty raising their arms to brush their hair or getting out of bed). Patients may also localise pain and tenderness to the neck muscles.
- Pelvic girdle pain - tenderness and signs of bursitis may be observed and, on observation, patients may have difficulty rising from a chair.
- Improvement in symptoms in one week with a trial of corticosteroids
- Systemic symptoms - low grade fever, fatigue, anorexia, weight loss and depression.
- Peripheral oligoarticular arthritis - wrists, knees and metacarpophalangeal joints may be affected at presentation
> > If the patient is younger than 50, an alternative explanation for the above symptoms should be sought as PMR is very rare in this age group.
When should PMR be referred to a rheumatologist?
- <60yrs
- Red flags of serious pathology (including weight loss, night pain or neurological features) but do not have any other identifiable cause for these
- Do not have the core symptoms of PMR (bilateral shoulder/pelvic girdle pain, >45 minutes of morning stiffness)
- Have unusual features of PMR (normal, or very high inflammatory markers, chronic onset of symptoms, limited response to steroids)
> > If patients present with PMR present with symptoms of GCA (headache, jaw claudication, visual disturbance) then urgent referral should be sought.
What are the investigations for PMR?
Baseline blood tests:
- CRP/ESR - both are usually is elevated at diagnosis.
The following would be expected to be normal in PMR but raised in the conditions listed:
- FBC - infections, leukaemia
- U&E - myeloma, renal failure
- LFT - infections, metastatic disease
- TSH - hypothyroidism
- Calcium - hyperparathyroidism, metastatic disease
- Creatine kinase - polymyositis, statin-induced myopathy.
- Serum protein electrophoresis - myeloma
- RF and anti-CCP - rheumatoid arthritis
- Other cancer biomarkers (e.g. PSA, CA-125) if there are features in the history suggestive of these conditions.
Additional tests recommended include:
- Urine dip - looking for haematuria as suggestive of cancer.
- Urinary Bence Jones protein - myeloma
- Chest x-ray - lung cancer, also needed as a baseline prior to considering methotrexate, which may be used in secondary care.
Imaging:
- Rarely needed and would rarely be requested in primary care. However, if the diagnosis is unclear, USS can be helpful to screen for bursitis with trochanteric bursitis being the most common bursitis and subacromial bursitis being associated with PMR.
Describe the diagnostic criteria for PMR
- Age >50 years
- Acute or subacute onset of symptoms representative of PMR
- Response to corticosteroids (prednisolone)
- Acute phase response - elevated CRP or ESR
- Exclusion of other conditions that mimic PMR - active infection, active cancer and GCA
What is the management of PMR?
Primary care management:
- Oral corticosteroids
- Gradually weaned off them with dose adjustments typically being every 4-8 weeks and reviews (telephone or face to face) scheduled for one week after each dose adjustment.
- Safety netting about the signs and symptoms of GCA and ensure patients know to seek urgent medical care if these develop.
Secondary care management:
- Patients should be referred if they have frequent relapses or are at higher risk of adverse effects from corticosteroid treatment
- Patients may be considered for DMARD treatment as 2nd line therapy (e.g. methotrexate) or tocilizumab as 3rd line.
What are the RFs for pseudogout?
haemochromatosis
hyperparathyroidism
acromegaly
low magnesium, low phosphate
Wilson’s disease
What are the clinical features of pseudogout?
- knee, wrist and shoulders most commonly affected
- joint aspiration: weakly-positively birefringent rhomboid-shaped crystals
What are the XR findings in pseudogout?
chondrocalcinosis - in the knee this can be seen as linear calcifications of the meniscus and articular cartilage
What is the management of pseudogout?
- Aspiration of joint fluid, to exclude septic arthritis
- NSAIDs or intra-articular, IM or oral steroids as for gout
What is psoriatic arthritis?
A chronic inflammatory arthritis which is associated with psoriasis.
It may be develop alongside the rash seen in psoriasis, or years later.
In rare cases it can be seen without a psoriatic rash.
What are the clinical features of psoriatic arthritis?
Psoriasis:
- Current or past psoriatic rash or nail changes
- Rash/nail changes usually precede joint involvement.
- Nail changes may be subtle, and can include pitting, ridges or onycholysis.
Inflammatory arthritis:
- Joint pain which is worse after long periods of rest, features prolonged morning stiffness of >30 minutes, and improves with activity.
- O/E signs of synovitis (tenderness and swelling of joints).
Features specific to psoriatic arthritis:
- Dactylitis (swelling of a whole digit) - NICE advise referral of anyone with dactylitis to a rheumatologist for assessment
- Enthesitis (inflammation at the site of tendon attachment) - commonly presents as elbow, heel or lateral hip pain
- Patients may report history of achilles tendonitis, plantar fasciitis or epicondylitis
What are the investigations for psoriatic arthritis?
> > Diagnosis often clinical
Diagnosis and management should be coordinated by secondary care, so referral to a rheumatologist should be made if psoriatic arthritis is suspected.
Investigations can be used to support diagnosis, or to help in differentiating between different forms of arthritis
Blood tests:
- Inflammatory markers (ESR/CRP) - often raised, especially in active disease
- RF - non-specific, so may be positive in up to 10% patients with psoriatic arthritis
- Anti-CCP - may be positive, more likely in severe disease
- HLA-B27 - positive result increases the probability of spondyloarthritis
Imaging:
- Plain film x-rays - changes may be absent in early disease, DIP joint erosion and periarticular new-bone formation, osteolysis and pencil-in-cup deformity in advanced disease/arthritis mutilans
- Ultrasound - helpful for diagnosis in absence of x-ray changes, tendon swelling, increased blood flow and erosions suggest inflammation
- MRI of affected joints may be considered
> > If psoriatic arthritis is confirmed, sacroiliac x-rays should be offered to look for evidence of asymptomatic involvement.
What is the management of psoriatic arthritis?
Conservative:
- Physiotherapy
- Hand therapy
- Podiatry
- Occupational therapy
> > Aim to assess their needs, provide advice/equipment, and regular follow-up
Medical:
- NSAIDs (NICE recommend using as adjunct to DMARDs)
- Intra-articular corticosteroid injections - alone or in combination with oral medication
- DMARDs considered if there is a failure of response to initial medical treatment or if there is severe disease at diagnosis
- 1st line: standard DMARDs (methotrexate, leflunomide or sulfasalazine)
- 2nd line: biological agents (etanercept, infliximab, apremilast)
- Short courses of oral corticosteroids may be needed while initiating DMARD therapy
Clinical features of Raynaud’s?
Triphasic colour change of the affected extremities:
- Pallor (white): This phase results from the initial vasoconstriction of arterioles, leading to reduced blood flow.
- Cyanosis (blue): As the oxygen supply in the tissues diminishes, a bluish discolouration occurs.
- Hyperemia (red): During the recovery phase, vasodilation leads to increased blood flow, causing a reddish hue.
Accompanied by symptoms:
- Numbness
- Cold sensation
- Tingling
- Pain
> > Episodes can last from minutes to hours and typically resolve upon rewarming or cessation of the triggering factor.
What are the investigations for Raynaud’s?
> > Essential to differentiate between primary and secondary Raynaud’s to guide treatment and management.
- FBC, ESR, CRP
- ANA
- RF and anti-CCP antibodies
- Cold-stimulus test
- Nailfold capillaroscopy
- Imaging studies, such as Doppler ultrasonography, to evaluate for occlusive arterial disease.
Management of Raynaud’s?
Non-pharmacological interventions:
- Patient education: Inform patients about the importance of avoiding triggers, such as cold exposure and emotional stress.
- Temperature regulation: Encourage patients to wear gloves, socks, and appropriate clothing to maintain warmth in cold environments.
- Smoking cessation: Smoking can exacerbate symptoms by causing vasoconstriction.
- Stress management: Teach patients stress reduction techniques, such as deep breathing exercises, meditation, and progressive muscle relaxation.
Pharmacological interventions:
- First-line = CCB e.g. nifedipine or amlodipine
- Second line = Alpha-blockers e.g. Prazosin and doxazosin
- Severe/refractory = Vasodilators e.g. Sildenafil, tadalafil, and prostaglandin analogs (e.g., iloprost)
Surgical interventions:
- Sympathectomy: In severe or refractory cases, a surgical sympathectomy may be performed to interrupt the sympathetic nerve supply to the affected extremity, promoting vasodilation and reducing vasospastic episodes. This approach should be reserved for cases that do not respond to conservative measures and pharmacological therapy.
- Revascularization procedures: In cases of secondary Raynaud’s with arterial occlusive disease, surgical or endovascular revascularization may be required to restore blood flow to the affected extremity.
What are the causes of reactive arthritis?
Usually 1-6w following gastrointestinal (Salmonella, Shigella Campylobacter) or urogenital pathogens (Chlamydia trachomatis)
What are the clinical features of reactive arthritis ?
MSK manifestations:
- Peripheral arthritis - acute asymmetric oligoarthritis (usually causing knee, ankle, and/or heel pain), Painful, swollen, warm, red, and stiff joints, especially in the morning
- Dactylitis (sausage digit) - painful inflammation of an entire finger or toe
- Enthesitis - inflammation of ligaments and tendons at the sites where they insert into bones, common sites are Achilles tendonitis presenting heel pain, plantar fasciitis, and pain at the tibial tubercle
- Axial arthritis -spinal inflammation, especially of the sacroiliac joints and lumbosacral spine, manifests as nonspecific low back pain and/or buttock pain and stiffness, especially during times of inactivity.
Extra‑articular manifestations:
- Ocular inflammation in the form of conjunctivitis can cause redness, tearing, and a sterile purulent discharge during the acute period.
- Anterior uveitis - pain, redness, and photophobia.
- Keratitis, corneal ulceration, episcleritis, retrobulbar neuritis, and anterior chamber haemorrhage may be present in chronic disease.
- Chlamydia infection cause genitourinary symptoms in 70-80% patients.
- In men, prostatitis is found in 80% of cases. But, urethritis and hemorrhagic cystitis can also occur.
- In women, non‑purulent cervicitis causing cervical bleeding, salpingo-oophoritis, or cystitis can occur.
- Yersinia infection causes mild diarrhoea
- Salmonella and Campylobacter infections can cause severe and of longer duration diarrhoea increasing the likeliness to develop ReA
- Colonoscopic findings in these patients resemble UC or CD
- Keratoderma blennorhagicum: hyperkeratotic skin on palms and soles which mimics pustular psoriasis
- Circinate balanitis: painless ulcers and plaque-like lesions on the shaft or glans of the penis
- Oral ulcers
- Nail changes like onycholysis, subungual keratosis, or nail pits
- Constitutional symptoms: malaise, fever, fatigue, and weight loss are
- Cardiac manifestations: pericarditis, aortic disease, conduction abnormalities
What are the investigations for reactive arthritis?
> > Clinical diagnosis
Blood tests:
- Elevated ESR and CRP
- RF and ANA - rule out other forms of arthritis
- HLA‑B27 testing: non-diagnostic test, but positive in around 40% of patients. It is useful as a prognostic marker of ReA.
Evidence of preceding infection:
- Urinalysis may show increased leukocytes, hematuria, and mild proteinuria during acute disease.
- NAAT to detect Chlamydia trachomatis
- Stool test - Salmonella, Shigella, Campylobacter, and Yersinia
Evidence of inflammatory arthritis, enthesitis or synovitis:
- Radiography - in the early stage, there are no specific abnormalities except for soft tissue swelling.
- Radiographic changes are found in 70% of patients with long‑standing disease (chronic ReA).
- Erosive joint damage affects especially small joints of the feet
- Radiographic changes of sacroiliac joints, usually unilateral
- MRI: Helpful to assess enthesitis and involvement of sacroiliac joints, when radiographs may be negative
Synovial fluid analysis:
- Joint fluid (when possible) should always be examined to exclude alternative diagnoses like septic arthritis.
- Synovial fluid cell count is increased, with predominant neutrophils in the early stage of the disease.
- Synovial cultures are always negative.
What is the management of reactive arthritis?
Treatment of arthritis:
- NSAIDs: first-line therapy for acute phase
- Corticosteroids - during an acute flare, or unresponsive to NSAIDs.
- Intra-articular injections in mono-articular and oligoarticular forms, while systemic corticosteroids may be required if many joints are affected.
- DMARDs - second-line agents either when NSAIDs and CS fails or for those who develop chronic or erosive reactive arthritis
- Sulfasalazine is effective in peripheral disease and has little or no effect on spinal disease.
- Methotrexate is effective in treating both acute and chronic ReA especially, in patients with spinal involvement.
- Anti-TNF-α therapy - if symptoms persist after all other treatments exhausted. Etanercept is an emerging therapy, effective in treating chronic ReA on a small series of cases.
- Physiotherapy - may help relieve symptoms by increasing muscle strength and improving mobility and function
Antibiotics:
- Acute Chlamydia genitourinary infection or with previously documented but untreated infection, but not for patients with established reactive arthritis
- Antibiotics are generally not indicated for uncomplicated gastrointestinal infections.
- Oral regimens of either doxycycline or azithromycin, both combined with rifampin for 6 months have shown significant symptom remission
Treatment of other manifestations:
- Circinate balanitis and mild to moderate keratoderma blennorrhagica should be treated with topical steroids.
- Oral lesions resolve spontaneously and require no treatment.
- Eye lesions, especially uveitis, should be managed with ophthalmologic advice.
- Systemic corticosteroids can be helpful in treating ocular manifestations, such as anterior uveitis.
What are the clinical features of systemic sclerosis?
Skin:
Thickening, tightening, and hardening of the skin, which typically starts in the fingers and progresses proximally.
Raynaud’s phenomenon:
Vasospasm-induced color changes (pallor, cyanosis, and erythema) in the fingers and toes in response to cold or stress.
Gastrointestinal involvement:
Dysphagia, gastroesophageal reflux, and motility disorders affecting the esophagus, stomach, and intestines.
Pulmonary involvement:
Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are common complications, which can cause shortness of breath, cough, and chest pain.
Renal involvement:
Scleroderma renal crisis, characterized by rapidly progressive renal failure and malignant hypertension, is a life-threatening complication of SSc.
Musculoskeletal involvement:
Joint pain, stiffness, and contractures due to fibrosis of the joint capsules and tendons.
