Rheumatology Main风湿病 21-30

Q2
A 52~year-old woman comes to the physician with a rash over her face that began a few weeks ago. Lately, the patient has also been having difficulty rising from a seated position and climbing stairs. On examination, an erythematous rash on the upper chest and violaceous periorbital edema are present. Her vital signs are within normal limits. Examination shows symmetric proximal muscle weakness in the legs. This patient's condition is most often associated with which of the following? A.Alveolar hemorrhage B.Aortic aneurysms C.Carpal tunnel syndrome D.Glomerulonephritis E. Malignancy F. Sudden visual loss
A1
Correct answer: C The patient described most likely has De Quervain tenosynovitis, a condition that classically affects new mothers who hold their infants with the thumb outstretched (abducted / extended). This condition is caused by inflammation of the abductor poliicis longus and extensor poliicis brevis tendons as they pass through a fibrous sheath at the radial styloid process. Tenderness can typically be elicited with direct palpation of the radial side of the wrist at the base of the hand. Additionally, the Finkelstein test, which is conducted by passively stretching the affected tendons by grasping the flexed thumb into the palm with the fingers, elicits pain. (Choice A) Osteoarthritis of first metacarpophalangeal joint would cause pain with activity over the first metacarpophalangeal joint, not at the carpometacarpal joint. (Choice B) Trigger thumb results in pain over the palmar aspect of the first metacarpophalangeal joint and locking of the thumb in flexion.
(Choice D) Scaphoid fractures typically occur during forceful hyperextension of the wrist as may occur in a fail on an outstretched hand. Pain typically localizes to the anatomic snuffbox. (Choice E) Flexor carpi radialis tenosynovitis results in pain with radial flexion of the wrist and point tenderness over the trapezium.
A2
Correct answer: E This woman with proximal muscle weakness and rash has features consistent with dermatomyositis. Dermatomyositis is an autoimmune condition that is 6 times more common in women. In its most classic form, it causes a proximal extensor muscle inflammatory myopathy and characteristic cutaneous findings (eg, violaceous poikiloderma over various regions of the body). On the face, the eruption is most often accompanied by periorbital edema and is known as the heliotrope sign. On the chest and lateral neck it is called the shawl sign. On the knuckles, elbows, and knees it is referred to as Gottron's sign. Violaceous, slightly scaly papules overlying the joints are known as Gottron's papules; these are pathognomonic for dermatomyositis. Patients have an elevated creatine phosphokinase (CPK) that is generally 10 times the upper limit of normal. The classic autoantibodies associated with dermatomyositis are anti-Jo-1 (antisynthetase antibody) and anti-Mi-2 (against heiicase). internal malignancies are more common In patients with dermatomyositis (>15% of adult patients) compared to the general population. The most common malignancies are ovarian, lung, pancreatic, stomach, or colorectal cancers, and non-Hodgkin lymphoma. Regular, age-appropriate cancer screening is essential In these patients. (Choice A) Inflammatory diseases associated with alveolar hemorrhage include Goodpasture syndrome, granulomatosis with poiyangiitis (formerly Wegener's), microscopic poiyangiitis, eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), Behcet syndrome, systemic lupus erythematosus, and antiphospholipid antibody syndrome. Dermatomyositis can cause pulmonary fibrosis in roughly 10% of patients. (Choice B) Inflammatory diseases associated with aortic aneurysms Include Behcet syndrome. Takayasu arteritis, giant cell arteritis, ankylosing spondylitis, rheumatoid arthritis, psoriatic arthritis, relapsing polychondritis, lgG4-related disease, and reactive arthritis. (Choice C) Inflammatory or depositional diseases associated with carpal tunnel syndrome include rheumatoid arthritis, sarcoidosis, hypothyroidism, and amyloidosis. (Choice D) Autoimmune diseases associated with glomerulonephritis include systemic lupus erythematosus, Goodpasture syndrome, relapsing polychondritis, cryoglobulinemia, granulomatosis with polyangiitis (formerly Wegener's), and microscopic poiyangiitis, among others. It is not common for the elevated CK in dermatomyositis to cause a myoglobin-induced acute kidney injury, but when it does it is due to direct injury to the tubular cells rather than to glomerulonephritis. (Choice F) Autoimmune diseases associated with sudden visual loss include temporal arteritis, multiple sclerosis, and, rarely, systemic lupus erythematous. Educational objective: Dermatomyositis is characterized by classic cutaneous findings accompanied by proximal muscle weakness. Over 15% of adult patients will have or develop an internal malignancy, most commonly ovarian, lung, pancreatic, stomach, or colorectal cancers, or non-Hodgkin lymphoma. Regular, age-appropriate cancer screening is essential in these patients.