Case (19)

[Kassim]

Yusuf is a  38 year old  male with hypertension presented with one day of headache, chest pain, dyspnea and six months of progressive leg weakness, dysphagia and post-prandial vomiting. Upon specific questioning, he also endorsed cold induced pain in his fingers. Exam revealed a thin young man with minimal facial expression. His blood pressure was 200/110. He had  telangiectasia around nasolabial folds. He had papilledema with AV nicking and an S4 gallop. He had purple atrophic fingertips and thickened skin in the extremities. He had edematous lower extremities with 4–/5 proximal muscle strenth. Labs revealed BUN 42, Cr 5.7, Hb 9.5. His cardiac enzymes were negative. His urine showed protein >300 mg/dl, 4–10 RBC. Barium swallow revealed decreased motility.
Echocardiogram showed LV hypertrophy, EF 45%, and increased pulmonary artery pressure. Subsequently, a kidney biopsy demonstrated severe arteriolosclerosis suggestive of malignant hypertension, as well as focal segmental glomerulosclerosis.
The patient was discharged on with medication
What is the Dx
What further investigation would you order to confirm the Dx.
what are the medication

[Diagnostic]

Another interesting complicated case.
This guys is having some sort of generalized thinkening & I think it's scleroderma because of:
1- Painful atrophic fingertips (raynaud's phenomenon)
2- Thickened skin in the extremities
3- Talengectasia (scelorosing telengectasia)
4- Dysphagia + Post-prandial vomiting + decreased motility may be due to GIT thickening.
5- Also this thickening involved lungs (chest pain & dyspnea), Heart (walls and vulvular thickening - LV hypertrophy), Muscles (leg weakness) & Kidneys (severe glomeruscelorosis leading to severe hypertension).

To confirm scleroderam we need positive anti-nuclear antibody test and the goal of treatment is to control the hypertension, maintain kidney fuction because no treatment for this autoimmune disease athough cyclosporin found beneficial to decrease autoimmunity.
Diagnostic

[Kassim]

 yes, that is the correct answer.

[Admin]

Congratulations
The 15 points of this case goes to Diagnostic
Dr.Mahdi