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just 4 you - pursuit
#1


A 62-year-old man is evaluated in the emergency department for a 1-month history of nonproductive cough, progressive dyspnea on exertion, fever, arthritis, and weakness. He has had increasing difficulty rising from a chair, climbing stairs, and holding his arms up to comb his hair. For the past week, he has noticed pain and color changes of his digits when exposed to the cold. His medical history is otherwise unremarkable, and he takes no medications.

On physical examination, temperature is 38.7 °C (101.6 °F), blood pressure is 148/88 mm Hg, pulse rate is 100/min, and respiration rate is 34/min. Pulse oximetry shows 92% oxygen saturation with the patient breathing 2 L/min of supplemental oxygen. There is no jugular venous distension. Cardiac examination reveals normal heart sounds without extra sounds, murmurs, or rubs. On pulmonary examination, late bilateral fine crackles are noted over the lower half of the lung fields. The sides of the fingers appear rough and cracked. He has tenderness and synovial thickening involving the wrists and the second and third metacarpophalangeal joints. Grip strength is intact, but he has evidence of weakness in the muscles of the upper arms and legs and neck flexors.

Laboratory studies:

Hemoglobin 13.8 g/dL (138 g/L)

Leukocyte count 10,600/µL (10.6 × 109/L)

Creatine kinase 5400 U/L

Antinuclear antibodies Titer of 1:640

Anti“double-stranded DNA antibodies Negative

Anti-Smith antibodies Negative

Anti“Scl-70 antibodies Negative

Anti“Jo-1 antibodies Positive

Chest radiograph is shown: http://yfrog.com/mdcaptureklp

Which of the following is the most likely diagnosis?

A-Antisynthetase syndrome

B-Idiopathic pulmonary fibrosis

C-Systemic lupus erythematosus

D-Systemic sclerosis
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#2
Perip ms weakness, high cpk
Anti“Jo-1 antibodies Positive.....Polymyositis

ans.. A
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#3
that is right
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#4
WOW, what is that antisynthetase syndrome ?
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#5
I didn't know either, amateur just like youSmile
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#6
Pursuit, explaination please!
Can you post 2 Q of Acid base ..for me tomorrow,if you don't mind, i am reading that topic right now .
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#7
Thanks
Smile
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#8
I have GI on schedule tomorrow but I will if I have any Acid-base

Explanation


This patient most likely has the antisynthetase syndrome. Polymyositis and dermatomyositis are inflammatory myopathies that typically present with the gradual onset of symmetric proximal weakness over weeks to months. This weakness causes progressive difficulty with rising from a chair, climbing stairs, or hair combing. Nearly one third of patients with an inflammatory myopathy have the antisynthetase syndrome. This condition can have an acute or subacute onset and is characterized by fever; fatigue; Raynaud phenomenon; synovitis; interstitial lung disease; and scaly, rough, dry, darkened, cracked horizontal lines that develop on the palmar and lateral aspects of the fingers that are known as œmechanic™s hands.

Patients with antisynthetase syndrome have antisynthetase antibodies that are specific for an inflammatory myopathy. The most common antisynthetase antibody is the anti“Jo-1 antibody. Not all patients with anti“Jo-1 antibodies develop the antisynthetase syndrome and not all patients with this syndrome have all of its manifestations, but patients who have anti“Jo-1 antibodies typically have several features of the antisynthetase syndrome in addition to their muscle disease. The greatest concern in patients with this syndrome is the risk for developing interstitial lung disease.

Idiopathic pulmonary fibrosis (IPF), the most common of the idiopathic interstitial pneumonias, is a fibrosing interstitial pneumonia. This condition manifests as slowly progressive dyspnea and a chronic, nonproductive cough. Pulmonary examination in patients with IPF reveals end-inspiratory crackles, and chest radiograph performed on presentation nearly always reveals decreased lung volumes and basal reticular opacities. However, IPF is not associated with extrapulmonary involvement.

Patients with systemic lupus erythematosus (SLE) may have fever, arthralgia or arthritis, Raynaud phenomenon, and very rarely, an acute interstitial pneumonia (lupus pneumonitis). However, SLE usually affects women in their 20s and 30s and is often associated with additional manifestations such as serositis, aphthous ulcers, malar or photosensitive rash, and specific autoantibodies including anti“double-stranded DNA and anti-Smith antibodies. SLE is an unlikely diagnosis based upon the extent of muscle weakness, degree of elevation of the creatine kinase level, absence of specific antibodies, and presence of anti“Jo-1 antibodies.

Systemic sclerosis most frequently develops in patients between 30 and 50 years of age and has a 3:1 female predominance. Diagnosis may be established in patients who present with two of the following features: sclerodactyly, digital pitting, and basilar fibrosis visible on chest radiography. Antinuclear antibodies are present in more than 95% of patients with systemic sclerosis. Patients with anti“Scl-70 antibodies are at increased risk for developing diffuse cutaneous disease and interstitial lung disease. Systemic sclerosis is unlikely in the absence of sclerodactyly or digital pitting. Furthermore, this condition would not explain this patient™s proximal muscle weakness.


check the picture bank link for mechanic™s hands:
http://images.rheumatology.org/search.ph...hField=ALL&searchstring=mechanic%92s+hands
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#9
Thanks for posting. I got it. I just guess and pick up. i don't know either .GN.
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