Need to know rheum Flashcards
What are the antibodies seen in and presentation associated with Limited cutaneous systemic sclerosis?
Anticentromere antibody in 50-60% of cases
Minimal, some dysphagia or heartburn
Skin changes: Hands, proximal forearms, feet, face only
Present for years prior to diagnosis (often 10+) often with telangiectasias
what are the antibodies seen with and presentation associated with diffuse cutaneous systemic sclerosis
Anti-Scl-70 and anti-RNA polymerase antibodies
Early renal, lung, GI, and myocardial disease
Also often truncal and skin involvement
Presents close to skin changes and diagnosis (within 1 year)
What is Sine Scleroderma?
systemic sclerosis sine scleroderma (findings of organ involvement and antibodies without skin changes)
Antibodies are: Any antinuclear antibody pattern may be present (anti-Scl-70, ACA, or anti-RNA polymerase)
What Systemic sclerosis with overlap syndrome?
systemic sclerosis with overlap syndrome (SSc overlaps with systemic lupus, rheumatoid arthritis, polymyositis, or Sjögren’s syndrome)
What is Behcet’s Syndrome? Treatment?
Behçet syndrome is a multisystem disorder presenting with recurrent oral and/or genital ulcerations, chronic relapsing uveitis that may cause blindness, and neurologic impairments. The cause is unknown though it is primarily classified by auto-inflammation of the vessels. Diagnostic criteria established by the International Study Group for Behçet syndrome requires the presence of recurrent oral ulceration in the absence of other clinical explanations and two of the following: recurrent genital ulceration, eye lesions, skin lesions, and/or a positive pathergy test (pustular reaction of the skin in response to intradermal needle prick).
Treatment of Behçet syndrome aims to reduce inflammation, ease symptoms, and control the immune system. Corticosteroids have a suppressive effect on most manifestations of Behçet syndrome and are the treatment of choice for acute presentations. Anti-TNF medications such as etanercept or infliximab may be helpful for patients with primarily skin or mucosal manifestations. Other medications such as colchicine or thalidomide may also be used in severe cases. Intravenous immunoglobulin (IVIg) may also be used to treat severe cases
All patients on hydroxychloroquire get what screening?
It is recommended that all patients started on hydroxychloroquine get baseline visual field testing and continue regular screenings with ophthalmology.
Best testing for Myasthenia Gravis?
In myasthenia gravis, single-fiber electromyography testing of the frontalis muscle can be performed to establish diagnosis with good sensitivity and specificity.
Additionally, if disease is purely ocular, single-fiber electromyography is superior to the acetylcholine receptor antibody test in diagnosing this condition.
What medications can make Myasthenia Gravis worse?
Although the most common cause of exacerbation of MG is infection, an important distinction is the role of medications exacerbating disease as well. Among these are innumerable antibiotics including macrolides, fluoroquinolones, and penicillins. Nonantibiotic medications frequently implicated include beta-blockers, calcium channel blockers, gabapentin, and importantly, corticosteroids.
The latter is of significant importance because although corticosteroids are often a part of the treatment for MG flares, paradoxical worsening may occur in half of all patients with MG, and myasthenia crisis occurs in up to 18% of these patients. Therefore, among patients with MG, corticosteroid administration should always be administered in the hospital setting.
Dermatomyosistis features and labs?
This patient is presenting with classic findings of dermatomyositis, including progressive proximal muscle weakness associated with falls, constitutional symptoms (fever, fatigue and weight loss), myalgias, and a heliotrope rash (rash around the eyelids). The most commonly elevated enzymes in cases of dermatomyositis are muscle enzymes, mainly creatine kinase and aldolase.
The primary clinical feature of dermatomyositis and polymyositis is proximal muscle weakness. While the weakness is nearly always present at diagnosis in polymyositis, it can present after the skin findings in dermatomyositis. The characteristic skin findings of dermatomyositis are beyond the scope of this question but are covered in other areas of this question bank.
Creatinine kinase and aldolase are both present in muscle and serum levels rise secondary to muscle breakdown. Less than 5% of cases of dermatomyositis will have normal creatinine kinase or aldolase. Creatine kinase, in particular, has been shown to be the most sensitive and specific enzyme for this disorder. Several other laboratory values can be abnormal such as antinuclear antibodies (ANA), aspartate aminotransferase, lactate dehydrogenase (LDH), and myositis-specific antibodies (MSAs), such as anti-Mi-2 and anti-Jo-1, but they are not specific to dermatomyositis and may not be elevated in up to 20% of cases.
Antibody for SLE?
SLE Anti-dsDNA Specific for lupus
Drug induced Lupus antibody? feature of this?
Drug-induced SLE Anti-histone Specific for drug-induced lupus but can also be found in native SLE
Antiphospholipid antibody? Also seen with?
Antiphospholipid syndrome (APS) Antiphospholipid antibodies (anticardiolipin or anti-beta2-glycoprotein) Also seen in SLE without APS
Sjogren Syndrome Antibodies? Seen in?
Sjogren syndrome Anti-Ro and anti-La Seen in SLE and scleroderma as well
Limited Scleroderma aka? antibody?
Limited scleroderma (CREST) Anti-centromere Can also be seen in diffuse scleroderma
Diffuse scleroderma Anti-Scl-70 Can also be positive in limited scleroderma
Inflammatory myopathy (polymyositis) Anti-Jo-1 Specific for polymyositis
Mixed connective tissue disease (MCTD) Anti-snRNP70 (anti-U1 RNP) Can also be seen in scleroderma, SLE, and polymyositis
Diffuse Scleroderma antibody? positive also in?
Diffuse scleroderma Anti-Scl-70 Can also be positive in limited scleroderma
