WBR255

Author	[[PageAuthor::Gerald Chi (Reviewed by Yazan Daaboul, M.D.)]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Pathology
Sub Category	SubCategory::Musculoskeletal/Rheumatology
Prompt	[[Prompt::A 48-year-old woman visits the clinic for evaluation of her skin conditions. She has patches of hardened skin on her face, elbows, and knees. She feels that her fingers are tight especially in cold weathers, and become pale and cyanotic. She also complains of difficulty swallowing of both solids and liquids and often regurgigates her food. Laboratory findings are remarkable detection of nucleolar pattern of anti-nuclear antibodies as well as elevated anti-centromere antibodies. Which of the following conditions is most likely to be associated with this patient's condition?]]
Answer A	AnswerA::Renal tubular acidosis
Answer A Explanation	AnswerAExp::CREST syndrome is not typically associated with renal tubular acidosis.
Answer B	AnswerB::Primary sclerosing cholangitis
Answer B Explanation	AnswerBExp::CREST syndrome is not typically associated with primary sclerosing cholangitis.
Answer C	AnswerC::Libman-Sacks endocarditis
Answer C Explanation	AnswerCExp::CREST syndrome is not typically associated with Libman-Sacks endocarditis.
Answer D	AnswerD::Membranous glomerulonephritis
Answer D Explanation	AnswerDExp::CREST syndrome is not typically associated with membranous glomerulonephritis.
Answer E	AnswerE::Pulmonary hypertension
Answer E Explanation	AnswerEExp::CREST syndrome is associated with pulmonary hypertension.
Right Answer	RightAnswer::E
Explanation	[[Explanation::Systemic sclerosis is usually classified by the extent of cutaneous manifestations and may be either limited (70%, less severe - positive anti-centromere antibodies) or diffuse (30%, more severe with visceral manifestations - positive anti-Scl-70 antibody). The limited cutaneous form of systemic sclerosis (lcSSc) may include characteristic vascular manifestations and is then termed CREST syndrome which is an acronym for the five main features: Calcinosis, Raynaud's syndrome, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. The patient is most likely diagnosed with CREST syndrome, whereby she complains of hardened skin patches (calcinosis), tight pale fingers (Raynaud's phenomenon), difficulty swallowing (esophageal dysmotility), and has positive anti-centromere antibodies. The majority of patients with CREST syndrome do not have all 5 characteristic features of the disease, but often have variable manifestations. The association between CREST syndrome and vascular abnormalities predisposes to the development of pulmonary hypertension when the pulmonary vasculature is affected. While limited sclerosis is associated with pulmonary hypertension, the diffuse form is typically associated with pulmonary fibrosis. Educational Objective: Systemic sclerosis is usually classified by the extent of cutaneous manifestations and may be either limited (70%, less severe - positive anti-centromere antibodies) or diffuse (30%, more severe with visceral manifestations - positive anti-Scl-70 antibody). Vascular involvement in limited forms of scleroderms is referred to as CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia). CREST syndrome may be associated with pulmonary hypertension. References: Lonzetti LS, Joyal F, Raynauld JP, et al. Updating the American College of Rheumatology preliminary classification criteria for systemic sclerosis: addition of severe nailfold capillaroscopy adbnormalities markedly increase the sensitivity of limited scleroderma. Arthritis Rheum. 2001;44(3):735. Silman AJ. Scleroderma. Bailleres Clin Rheumatol. 1995;9(3):471-82. First Aid 2015 page 436.]]
Approved	Approved::Yes
Keyword	WBRKeyword::Systemic sclerosis, WBRKeyword::CREST syndrome, WBRKeyword::Pulmonary hypertension
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