Rheumatology [OTHR CONNECTIVE TISSUE DZ]

Scleroderma Sclerodactyly
Scleroderma is an autoimmune disease resulting in 1) collagen
replacing smooth muscle and 2) wide-spread extraneous Restrictive Esophageal dysmotility
Pericarditis (really bad GERD)
collagen deposition. The typical patient has skin tightness in the
hands and face and may suffer some GERD or Raynaud’s. In
reality, there are two types of scleroderma: CREST and Systemic
Sclerosis. CREST is a limited disease that spares the heart and Renal Failure and
kidneys but has Skin and GI effects. On the other hand, systemic Renovascular HTN Sclerodactyly
sclerosis is a diffuse disease affecting the trunk with cardiac
(restrictive cardiomyopathy) and Renal (renovascular
hypertension) effects. Scleroderma is mainly a clinical diagnosis
but antibodies may aid getting there. Anti-Scl-70 (topoisomerase CREST: Systemic Sclerosis
I) is positive in systemic disease while anti-centromere is Calcinosis CREST +
positive in CREST. There’s also no treatment for the disease Raynaud’s Renal
itself. Instead, treat the symptoms. Use Calcium Channel Esophageal Dysmotility Heart
Blockers for Raynaud’s, Penicillamine for skin changes, Sclerodactyly
steroids for acute flares, and aggressive treatment of Telangiectasia
hypertension with ACE-inhibitors. Collagen in the renal
arterioles prevents dilation and constrict. This creates a prerenal Anti-Centromere Anti-Scl-70
picture by activating the renin-angiotensin-aldosterone axis, PAH ILD
which exacerbates the hypertension.

Sjogren’s syndrome
An inflammatory condition of exocrine glands due to Dry Eyes
lymphoplasmacytic infiltration. It can exist on its own
(primary) or be part of another Rheumatologic disorder Parotid Glands
(secondary). It’s easy to spot: Dry Eyes (Keratoconjunctivitis), Dry Mouth
Dry Mouth (Xerostomia), and bilateral parotid enlargement.
It’s a clinical diagnosis assisted by antibodies (Ro or La which
are specific but not sensitive) and tear production testing
(Schirmer test). There’s no treatment so focus on symptom Heliotrope Rash
control (artificial tears / saliva).
Myocarditis Gottron’s Papules
Polymyositis / Dermatomyositis / Inclusion Body Myositis
These three inflammatory myopathies are lumped together
because their presentation, diagnosis, and treatment are all the Diaphragmatic
same (well, except IBM, which has no treatment). The underlying Weakness
pathogenesis (T cell / Immune Complex / T cell respectively)
results in the presentation of painful proximal muscle weakness Inflammatory
(difficulty in rising out of a chair but intact grip strength) Myopathy
occurring slowly over time. There may be systemic involvement.
The way it comes to light is usually the dermatologic signs: 1)
erythematous rash on sun-exposed areas (photosensitivity), 2) Scleroderma Sjogren’s DM/PM/IBM
Heliotrope rash, a purple discoloration around the eyes with Pt Female, Thickened Skin Female Proximal Muscle
periorbital edema, and 3) Gottron’s Papules (pathognomonic), on fingers, GERD, Dry eyes weakness, tender,
which are scaly areas symmetrically over major joints (wrists, Reynaud’s Dry mouth Heliotrope rash,
elbows, knees, ankles). The first test is always EMG to determine Bi Parotitis Gottron’s Papules
if it’s a nerve conduction issue (MS, for example) versus F: DM +PM, M: IBM
Ab Scl-70 = Diffuse Ro Jo
muscular damage. A muscle biopsy is done to definitively
Centromere = CREST La Mi
diagnose and separates one disease from the other. Other tests Dx - Schirmer EMG
may help. The CK will be elevated (muscular damage) and Anti- Tear Production Bx
Jo or Anti-Mi antibodies may be present. The goal is to check MRI / CT (Cancer)
for occult malignancy (as these are often a paraneoplastic Tx Penicillamine Symptom Relief Check for Malignancy
syndrome) and treatment is with high-dose steroids. CCB
Steroids High Dose Steroids
ACE

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