A 27-year-old man presents with 6 months of worsening low back pain. The pain is worse in the morning and associated with morning “stiffness” that improves with walking and exercise. The patient feels well and denies any systemic symptoms. Physical examination reveals limited range of motion on forward flexion of her lower back. X-ray reveals the presence of a “bamboo spine.
Which of the following is the most common extra-articular manifestation of this patient’s condition?
Anterior uveitis. The patient in this question is presenting with signs, symptoms, and radiologic findings consistent with a diagnosis of ankylosing spondylitis (AS). AS has a strong association with HLA-B27. The most common extra-articular manifestation of AS is anterior uveitis: inflammation in the anterior chamber of the eye. This can cause erythema, pain, and photophobia. The reason for this association is that both AS and uveitis share an inheritance with the HLA-B27 antigen. (A, B) Both aortic valve insufficiency and lung fibrosis are less commonly associated with AS. (D) Oral ulcers are associated with systemic lupus erythematosus (SLE) and Crohn disease, but not associated with AS.
A 37-year-old woman with a history of Crohn disease presents for follow-up. She has been taking prednisone for the last 4 years at varying dosages and now has worsening left hip pain. The pain is aggravated during weight-bearing activities and is not relieved by rest. Physical examination is significant for a blood pressure of 148/82 mmHg and limited range of motion of her left hip. Dermatologic examination reveals purple striae and thinning of her skin in the lower abdominal region. Neurologic examination is within normal limits. Laboratory values (including ESR and TSH) are within normal limits.
Which of the following is the underlying mechanism of this patient’s left hip pain?
Interruption of bone vasculature. This patient is steroid-dependent and is presenting with signs and symptoms consistent with a diagnosis of avascular necrosis, also known as osteonecrosis. This condition results when there is necrosis of bone components due to interruption of the blood supply. Avascular necrosis manifests with progressive pain with a limited range of motion. Risk factors include chronic corticosteroid use, alcoholism, hemoglobinopathies (e.g., sickle cell disease), HIV, and chemotherapy. The best and most sensitive test for avascular necrosis is MRI. (A) Cartilage erosion occurs in osteoarthritis. (C) Excessive fluid production from inflamed synovium describes an inflammatory arthritis such as rheumatoid arthritis. (D) Peripheral nerve damage can occur in diabetic patients and results in Charcot joint, which manifests as a deformed foot that develops from an inability to detect pain, proprioception, and temperature.
A 37-year-old woman presents with progressive shortness of breath. The patient also reports painful joints over the past 5 months, especially in her fingers. She has a past medical history of GERD, which is controlled with omeprazole. On physical examination, the patient has thickened skin over the acral surfaces. Examination reveals the following figure.
Which of the following will likely be positive in this patient?
Anti-topoisomerase antibodies. This patient likely has a diagnosis of systemic sclerosis, an autoimmune and connective tissue disease that is characterized by thickening of the skin from accumulation of collagen. There are two forms of systemic sclerosis: limited cutaneous scleroderma (i.e., CREST syndrome, which is limited to the skin on the face, hands, and feet) and diffuse cutaneous scleroderma (covers more of the skin and commonly involves the visceral organs such as the kidneys, heart, lungs, and gastrointestinal tract). Importantly, “CREST” refers to the five main features: Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. CREST syndrome is characterized by the presence of anti-centromere antibodies. Our patient is presenting with lung findings and therefore likely has the diffuse form of systemic sclerosis. This condition is characterized by the presence of anti Scl-70 antibodies (also called anti-topoisomerase I antibodies).
(A) Anti-histone antibodies are found in drug-induced lupus. Medications that are most likely responsible include hydralazine, procainamide, and isoniazid. (B) Rheumatoid factor (RF) is often found in rheumatoid arthritis but can also be found in a plethora of other autoimmune diseases. (D) Positive c-ANCA is found in granulomatosis with polyangiitis (Wegener granulomatosis).
A 47-year-old woman presents with diffuse joint pain over the past 5 days. Although the pain is diffuse, it is worst in the hand and wrist joints. The pain is at its worst in the morning and typically can take up to 20 minutes to reach the point where she can actually perform activities like walking and writing. The patient is otherwise healthy and denies fever, chills, weight loss, fatigue, or skin rashes. The patient reports that she is a third-grade teacher and has had several sick students over the past 4 months. Physical examination is unremarkable and no swelling or redness is noted over her joints. Laboratory values reveal a hemoglobin of 13.5 g/dL and an ESR of 18 mm/h.
Which of the following is the likely diagnosis in this patient?
Symmetric arthritis secondary to a viral infection. The patient in this question likely has viral arthritis from parvovirus B19 infection. Viral arthritis is distinguished from other types of arthritis by its acute onset of symmetric and polyarticular symptoms. Furthermore, it typically resolves entirely within 2 months and does not cause elevated inflammatory laboratory values (such as ESR). Our patient has only had symptoms for 5 days. The fact that she is in contact with children on a daily basis is a clue to the diagnosis as well. Although children typically present with the “slapped cheek” rash when infected by parvovirus B19, adults usually present with arthralgias typically affecting the finger and wrist joints.
(A) The diagnosis of fibromyalgia relies on tenderness in at least 11 out of the 18 “trigger” sites. (B) Rheumatoid arthritis (RA) is an inflammatory arthritis and would likely have an elevated ESR. Furthermore, RA typically presents with swelling, systemic symptoms, and morning stiffness lasting greater than 30 minutes. (C) Polymyalgia rheumatica presents with pain and/or stiffness in the shoulders and hips of an older patient. Moreover, the diagnosis is made only with a highly elevated ESR.
A 53-year-old man with a history of systemic lupus erythematosus (SLE) presents with hematuria. A renal biopsy demonstrates proliferative glomerulonephritis and the patient is started on cyclophosphamide.
Which of the following conditions is this patient at risk for developing with the initiation of this medication?
Hemorrhagic cystitis. Cyclophosphamide is a nitrogen mustard alkylating agent used to treat cancers and autoimmune disorders such as SLE and the vasculitides. With respect to SLE, cyclophosphamide is used primarily in patients with renal pathology. Side effects of cyclophosphamide include hemorrhagic cystitis, bladder cancer, and myelosuppression. Fortunately, patients can be administered mesna and encouraged to drink liberal amounts of fluids to prevent these complications. (A) Peripheral neuropathy is a toxic side effect of vincristine use. Other causes of peripheral neuropathy include phenytoin, alcohol, isoniazid, and antiretroviral therapy. (B) Hearing loss, specifically cochlear dysfunction, can be seen with cisplatin and carboplatin. Other causes of hearing loss include aminoglycosides. (D) Thyroid abnormalities can be seen with amiodarone (useful both in supraventricular arrhythmias and ventricular arrhythmias) as well as lithium (useful in the prophylactic treatment of bipolar disorder).
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