Connective tissue diseases (R4) Flashcards
In connective tissue diseases multisystem organ involvement is common, list 4 examples of this
What is SLE?
Multisystem autoimmune disorder with multiorgan involvement and a broad range of disease manifestation that vary over time
State the following about SLE:
- Who it primarily affects
- Peak age
- Ethnicitys at an increased risk
Primarily affects women of childbearing age M:F: 1:12
Peak incidence 15-45 years old
African, Caribbean and Chinese are at higher risk
What is the most common form of chronic SLE?
Discoid SLE
SLE presentation summary
- Fatigue, Wt loss, Fever
- Arthralgia, Myalgia and non-erosive arthritis
- Photosensitive malar rash
- Lymphadenopathy and splenomegaly
- SOB, Pleuritic chest pain
- Mouth ulcers, hair loss
- Raynaud’s phenomenon
Describe the typical Rash of SLE
Photosensitive malar rash - “butterfly” shaped across the nose and cheek bones
List 4 clinical musculoskeletal manifestations of SLE
- Non-erosive Arthritis
- Jaccoud arthropathy
- Arthralgia, Myalgia
- Myositis
List 4 dermatological manifestations of SLE
- Photosensitive malar rash
- Mouth ulcers
- Hair loss
- Cutaneous vasculitis (splinter haemorrhages/ purpura)
List 2 hematological manifestations of SLE
- Anemia of chronic disease (normocytic)
- Lymphopenia
Hemolyticanemia, Leukopenia, Thrombocytopenia, Pancytopenia
List 2 reticuloendothelial manifestations of SLE
Lymphadenopathy and Splenomegaly
List 2 pulmonary manifestations of SLE
- Pleural effusion
- Pneumonitis
ILD, Pulmonary hemorrhage, pulmonary hypertension, PE
List 3 cardiac manifestations of SLE
- Pericarditis (most common)
- Raynaud’s
- Myocarditis (arrhythmias and HF)
List 4 renal manifestations of SLE
Lupus nephritis - most common cause of SLE related death
ankle oedema, active sediment, hematuria, proteinuria, nephrotic syndrome, reduced eGFR
List 4 SNS and 4 PNS manifestations ‘neuropsychiatric SLE’
List 2 autoantibodies associated with SLE
State which is the most specific
- ANA
- anti-dsDNA (specific to SLE)
+ Anti; Smith, centromere, Ro, La, Scl70, Jo1
What 2 criteria allow a diagnosis of SLE to be made?
SLICC Criteria or the ACR Criteria
Explain the SLICC criteria
- At least 4 criteria incl at least one clinical AND one immunologic OR
- Biopsy proven lupus nephritis with positive ANA or Anti-DNA
First line treatment for SLE
- NSAIDs
- Steroids (prednisolone)
- Hydroxychloroquine (first line for mild SLE)
- Suncream and sun avoidance
What is Sjogren Syndrome?
A slowly progressive, inflammatory autoimmune disease affecting primarily the exocrine glands
Compare primary vs secondary Sjogrens syndrome
Primary: condition occurs in isolation.
Secondary: occurs related to SLE or RA
Pthophysiology of Sjogren Syndrome
Lymphocytic infiltration replaces functional epithelium leading to decreased exocrine secretions
Is Sjogren Syndrome more common in males or females?
What is the peak age affected?
F:M= 9:1
40-50 years old
List 3 clinical features of Sjogren Syndrome
- Fatigue
- Dry eyes, sensation of sand in eyes/ burning, redness of the eyes
- Dry mouth may be associated with difficulty eating and swallowing food
List 4 EXTRA-glandular manifestations of Sjogren Syndrome
With these manifestations what other disease must we always consider?
- Arthritis, arthralgia
- Skin : Raynaud’s phenomenon, palpable purpura
- Pulmonary: ILD, hilar lymphadenopathy
- Renal: interstitial nephritis, GN (rare)
- Neuromuscular: peripheral sensomotor neuropathy, cranial neuropathy
ALWAYS THINK LYMPHOMA!!
List 4 Laboratory findings of Sjogren Syndrome
- Anemia of chronic disease
- Lymphopenia
- Increased ESR, normal CRP
- ANA, Ro, La, RA
- Hypergammaglobulinemia
- Reduced C3, C4
Which antibodies are specifically associated with Sjogren’s syndrome?
Anti-Ro and Anti-La
List 3 diagnostic investigations for Sjogren Syndrome
- Salivary flow assessment: <1.5ml/15 min
- SCHIRMER TEST: <5mm in both eyes in 5 min
- Rose Begal staining: (stain of damaged epithelium)
Explain the Schirmer Test
Filter paper inserted under lower eyelid with strip hanging out for 5 minutes
Distance along strip that becomes moist is measured
Normally tears should travel 15mm, < 10mm is significant
List 4 treatments for Sjogren Syndrome
- Artificial tears
- Artificial saliva
- Vaginal lubricants
- Hydroxychloroquine is used to halt the progression of the disease
What is Scleroderma?
Autoimmune disease characterised by fibrosis across multiple organ systems and increased production of collagen in tissues
Also known as Systemic Sclerosis
Is scleroderma more common in males or females?
What is the peak age affected?
F:M: 4:1
45-60 y.o
What are the 4 criteria that classify scleroderma?
- SKIN THICKENING PROXIMAL TO THE MCP +
- Sclerodactyly
- Digital pitting scars
- Bibasilar lung fibrosis
What are the 2 types of Scleroderma?
Limited and Diffuse
What is Diffuse Cutaneous Systemic Sclerosis?
Subcategory of the scleroderma that affects multiple systems
Highest association with African Americans
What is Limited Cutaneous Systemic Sclerosis?
Subcategory of Scleroderma that affects fewer organs - sparing the kidneys and skin on the trunk and proximal limbs
Also known as CREST syndrome
List 5 features of limited cutaneous systemic sclerosis
(Hint: CREST)
- Calcinosis
- Raynaud’s phenomenon
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia
What antibody is associated with limited vs diffuse scleroderma?
Limited: Anti-centromere antibody
Diffuse: Anti-scl 70
List 3 initial features of systemic sclerosis
- Skin becomes tight
- Fingers become stiff and inflexible
- Symptoms of Raynauds
List 4 later features of systemic sclerosis
- Tightening of skin on the face and around lips
- Fatigue
- Joint and muscle pain
- Ulceration
- Vascular disease, Incl CAD
- Pulmonary fibrosis and PAH
- Renal failure (“scleroderma renal crisis”)
List 4 signs of systemic sclerosis
- Telangiectasia and calcinosis
- Barrnett’s sign: vertical striation with neck extension
- Mauskopf facies
- Salt and pepper (changes in pigmentation)
List 4 differences between limited vs diffuse scleroderma
What is Raynaud’s phenomenon
Episodic peripheral ischaemia in response to cold or emotion
Can be Primary or Secondary
Is Raynaud’s phenomenon more common in males of females?
F:M: 9:1
Explain the ‘triphasic reaction’ of Raynaud’s
Skin changes from white to blue to red
- Pallor: vasoconstriction of digital vessels
- Cyanosis: deoxygenation of static venous blood
- Red : reactive hyperemia due to reperfusion
Raynaud’s is most commonly associated with what disease?
SLE
First line management for Raynauds
Nifedipine (dihydropyridine CCBs)
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