Wednesday, May 21, 2014

1. Dermatomyositis
- Proximal muscle weakness
- Heliotrope rash
- Gottron papules: red-purplish plaques on the dorsal hands, more prominent over the joints
- Anti-Jo-1 antibodies associated with increased risk of ILD
- ILD: can present with the onset of myopathy or develop over time; interstitial opacities on CXR, interstitial pneumonia on high-res CT. Don't need a lung bx for diagnosis, but may need to bronch to r/o infection
- Dermato and polymyositis have a slightly higher risk than other rheumatic diseases (1-2% risk) of developing PCP on combo immunosuppression (incl steroids). Presentation: acute onset of fever and respiratory failure.
- Polymyositis unlikely without proximal muscle weakness and elevated CK
2. Fibromyalgia: 
- Diffuse pain on both sides of the body, above and below the waist, +/- tender points
- Often have fatigue and sleep disturbance, high rates of comorbid chronic fatigue syndrome, migraine, IBS, TMJ pain, pelvic pain.
3. ANA
- Can be elevated in general population, especially people with autoimmune thyroid disease or first-degree relatives of people with lupus.
- High titers do not necessarily indicate autoimmune disease.
4. Sjogen's
- Lymphocytic inflammation of lacrimal glands => no tears, keratoconjunctivitis sicca
- Lymphocytic inflammation of major/minor salivaary glands => xerostomia/salivary gland enlargement
- Can have many autoantibodies, including SSA/Ro or SSB/La but also ANA, RF, and hypergammaglobulinemia.
- Schirmer's test- stick a piece of filter paper in both eyes (after topical anaesthetic to prevent paper-irritation-induced tears) rely on capillary action to draw fluid into paper chromatography style. >15 mm in 5 minutes is normal, although in older adults (like the age group of sjogren's, 40-60s) about 1/3 may only be able to wet up to 10mm.
5. Polyarteritis nodosa
- Necrotizing inflammation of medium or small arteries
- Fever, msk symptoms, vasculitis
- Heart: affects coronaries, can cause HF 2/2 ischemia or HTN from renal effects
- Renal failure with proteinuria or hematuria. Kidneys are most common organ affected. Aneurysms in kidney can rupture spontaneously leading to perinephric hematomas, or they can be ruptured during attempted renal biopsy (so try to avoid biopsying the kidney). Generally you will see sub-nephrotic proteinuria and hematuria, but no red cell casts as there is no glomerular inflammation (only ischemia). If you see red cell casts, consider a different diagnosis like an ANCA+ GN or lupus.
- Nerves- mononeuritis multiplex-- affects up to 70% of people with PAN, polyneuropathy affecting named nerves like radial, ulnar, peroneal that starts asymmetric and expands to a confluent distal polyneuropathy
- GI: mesenteric angina picture, can worsen to the point of infarct and perforation
- Diagnose with sural nerve biopsy or kidney angiography
6. Wegener's - necrotizing vasculitis of upper/lower respiratory tract (alveolar hemorrhage, otitis media) and kidneys. Can cause arthritis and joint effusions of large and small joints. C-ANCA/antiproteinase 3 = 90% specific.
7. GCA- should initiate high dose steroids (1g/day or 100mg TID for 3 days) if suspected. Does not compromise results of temporal artery biopsy, recommend bx within 2 weeks (although positive results have been seen after 6 weeks). Bx yield higher when performed sooner.
8. ACS
- Stable angina:  angina that occurs predictably with a certain degree of exertion, relieved by nitroglycerin or rest
- Unstable angina; angina at rest, no biomarkers
- NSTEMI: significant angina at rest (or acutely worsening/different angina), with biomarkers but no ST elevation on EKG
- STEMI: clinical presentation for acute MI + ST elevation on EKG
- Typically, to be concerned enough to send someone for cath, you want at least 2/3 warning signs: either convincing clinical history, biomarkers, or significant EKG changes
- PCI + stent has better 30-day mortality vs tPA, and is preferred in cardiogenic shock and those with a CI to tPA (ischemic stroke w/in 3 mos, hx of intracerebral hemorrhage, suspected aortic dissection, bleed). PCI/stent within 12 hours, <90 minutes best.
9. Pericarditis
- Diagnose with 2/3 of the following: pleuritic chest pain (worse with supination or inspiration), friction rub (triphasic: heard during atrial contraction, ventricular contraction, and rapid ventricular filling), diffuse ST-segment elevation and PR depression
- May radiate to shoulder
10. Angina without coronary disease: what is the cause? 
 2013 Jul 15;167(1):168-73. doi: 10.1016/j.ijcard.2011.12.053. Epub 2012 Jan 10.

Invasive findings in patients with angina equivalent symptoms but no coronary artery disease; results from the heart quest cohort study.

BACKGROUND:

The cause of angina in patients presenting at coronary angiography without significant coronary artery disease (CAD) has not been systematically assessed in a large prospective cohort. This study is aimed to identify the cause of angina in these patients.

METHODS:

This prospective cohort comprised 718 consecutive patients with angina equivalent symptoms and no CAD (defined as no coronary stenosis ≥ 50%) between January 1st 1997 and July 31st 2008. All patients underwent additional invasive testing (intracoronary acetylcholine administration, fast atrial pacing). Small vessel and vasospastic diseases were diagnosed according to symptoms and vessel reaction during testing.

RESULTS:

Mean age was 56.3 ± 11.0 years (range 15 to 81 years). A majority of 431 patients (60.0%) had small vessel and/or vasospastic disease (233 patients had small vessel disease, 145 vasospastic disease and 53 a combination of both). Additional 87 patients (12.1%) had another cardiac disease. Only in a minority of 200 study participants (27.9%) that the symptoms were attributed to an extracardiac problem. Patients with small vessel disease were more likely to be female, to have hypertension, to have a family history of CAD and to have effort-related symptoms. Patients with vasospastic disease were more likely to be current smokers, to have angina at rest or to present as myocardial infarction, and to have coronary sclerosis and/or endothelial dysfunction.

CONCLUSIONS:

In a majority of patients with angina but no significant CAD, a cardiac cause of their symptoms can be found. Systematical invasive testing may help optimizing the medical management of these patients.

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