scleroderma Flashcards
diffuse and limited systemic sclerosis, localized syndromes, chemical/drug-related
What are the gastrointestinal manifestations of scleroderma?
Gastrointestinal manifestations of scleroderma include small oral architecture, Sjogren’s syndrome, reflux esophagitis/stricture, gastroparesis, pseudo-obstruction, bacterial overgrowth, pneumatosis intestinalis, colonic pseudo-diverticula, and sphincter involvement with fecal incontinence.
How common is dysphagia in scleroderma?
Dysphagia occurs in about 25% of patients with scleroderma, 85 – 90% have changes by manometry, and 60 to 70% by barium swallow.
The sensation of eye dryness correlates best with what corneal abnormality?
Dryness correlates best with increased friction of the upper lid moving over the cornea.
What changes occur early by MRI in the wrists of patients with scleroderma?
Lunate edema with effusions in the intercarpal joints and radio-carpal and ulnar-carpal joints.doi: 10.1186/ar4128
What do elevated platelet factor 4 plasma levels mean in systemic sclerosis?
Elevated plasma levels of platelet factor 4 (CXCL-4) correlate with pulmonary fibrosis and pulmonary hypertension in systemic sclerosis.
With systemic diseases can cause muscle enzyme elevation?
Systemic disorders such as inflammatory myopathy (polymyositis/dermatomyositis, systemic lupus erythematosus, and mixed connective tissue disease with antibodies to RNP), infectious myopathy, drug induced myopathies (alcohol, lipid lowering drugs, cocaine, antimalarial drugs, antipsychotic drugs, colchicine, antiretroviral drugs, ipecac, Interferon alpha and penicillamine), dystrophic myopathies, neuroleptic malignant syndrome, metabolic myopathies, hyperthermia, motor neuron disease, endocrine myopathy, and periodic paralysis should be considered. Presymptomatic myopathies as in the early stages of polymyositis, distal myopathy, mitochondrial myopathies, inclusion body myopathy, muscular dystrophy, myotonic dystrophy, and predisposition to malignant hyperthermia.
What is the usual initial treatment for polymyositis, and dermatomyositis?
Initial treatment of polymyositis, dermatomyositis, and inclusion body myositis are similar. A course of prednisone at 1 mg per kilogram (Max 80 mg) daily for six weeks followed by reduction (10 mg per week until 40 mg daily, then 5 milligrams per week until 20 mg daily, 2.5 mg per week until 10 mg daily, then 1 mg every two weeks until 5 mg daily). Responders may be off treatment by one year. Azathioprine at 2.5 mg per kilogram or methotrexate at 25 mg per week is usually used along with low-dose prednisone (5 to 15 mg daily).
What signs indicate prognosis in scleroderma?
387 US caucasian, Pittsburg: age at first visit(-1,0,1:55),skin thickness progression rate( 0 or 1, rapid), Gi severity (0,1,2: mild/mod, severe), anemia (0, 2: <11).
A&R 6/14, 1616
ANA negative patients (6%) less severe disease.
What is unusual about scleroderma fibroblasts?
Scleroderma fibroblasts resist apoptosis through multiple mechanisms including TGF-beta stimulation that interference with fas ligand. IL-6 and IL 17A inhibit pathways that utilize peroxisome proliferator-activated receptor gamma, Wnt, or hedgehog signaling.
What are some of the known causes of interstitial lung disease?
Known causes of interstitial pneumonia include silicates (silicosis, asbestosis, talk, cement, beryllium), carbon (coal dust, graphite), metals (tin, aluminum, hard metal dusts), iron, antimony, mixed, CuSO4, inhaled organic dusts (thermophilic fungi, bacteria, animal protein), gases (oxygen, oxides of nitrogen, sulfur), fumes,vapors, aerosols.
How is diffuse parenchymal lung disease subdivided?
Diffuse parenchymal lung disease is usually divided into 1) known cause, 2) IIP ( idiopathic interstitial pneumonia), 3) granulomatous DPLD (diffuse peripheral lung disease)-sarcoidosis, 4) other-LAM (lymphangiolieomyomatosis), PLCH.(pulmonary Langerhans’ cell histiocytosis).
How is idiopathic interstitial pneumonitis subdivided?
IIP is then subidivided into 1) chronic fibrosing (idiopathic pulmonary fibrosis , idiopathic nonspecific interstitial pneumonia, 2) acute/subacute fibrosing (cryptogenic organizing pneumonia, acute interstitial pneumonia), 3) smoking-related (respiratory bronchiolitis interstitial lung disease, desquamative interstitial pneumonia)
What is characteristic of nonspecific interstitial pneumonia?
Nonspecific interstitial pneumonia: cellular pattern-mild to moderate interstitial chronic inflammation, type II pneumocyte hyperplasia, no evidence of-dense interstitial fibrosis, severe alveolar inflammation, fibroblasts and foci with dense fibrosis, acute lung injury pattern (hyaline membrane), granulomas, viral inclusions, organisms
What is the trade-off between hematopoetic stem cell transplantation and intravenous Cytoxan when treating early diffuse scleroderma?
A randomized controlled trial of hematopoetic stem cell transplantation versus IV cytoxan in scleroderma showed increased treatment-related mortality in the first year (8,0)with significant long-term event free survival benefit. PMID 25058083
What antibodies occur in patients with digital ulcers?
In 2400 patients with digital ulcers 50% had localized scleroderma, 35% diffuse cutaneous scleroderma, 95% were ANA positive , 45% had SCL 70 antibodies, and 40% had anti-centromere antibodies. Ulcers appear early in diffuse scleroderma and/or anti-SCL 70 antibodies.
The presence of anti-centromere antibodies in patients with systemic sclerosis increases the risk of carotid plaques (67/40%) and ischemic arterial beds (32% versus 11%). doi 10.1186
