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Images in Clinical Medicine
Lingual Raynaud's Phenomenon
Figure 1.
- February 16, 2012
- Katada Y. and Tanaka T.
- N Engl J Med 2012; 366:e12
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A 20-year-old woman presented with a report of tongue numbness. The patient had a long-standing history of Raynaud's phenomenon and had received a diagnosis of mixed connective-tissue disease; testing was positive for antinuclear and anti-...
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Review Article
Mechanisms of Disease: IgG4-Related Disease
IgG4-related disease is a newly recognized fibroinflammatory condition characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum IgG4 concentrations. The disease was not recognized as…
- February 9, 2012
- Stone J.H., Zen Y., Deshpande V.
- N Engl J Med 2012; 366:539-551
A number of autoimmune diseases that affect diverse organ systems have recently been noted to be related to IgG4 autoantibodies. The authors review the spectrum of IgG4-related disease and the current status of diagnostic and management approaches.
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Images in Clinical Medicine
Tophaceous Gout
Figure 1.
- January 19, 2012
- Samaras N. and Rossi C.
- N Engl J Med 2012; 366:e6
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A 74-year-old woman with chronic renal failure was admitted for diarrhea and functional impairment. She was noted to have a tender, soft swelling of the medial and distal phalanx of the right index finger (Panel A). She had no history of joint ...
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Review Article
Mechanisms of Disease: Systemic Lupus Erythematosus
Although the term "lupus erythematosus" was introduced by 19th-century physicians to describe skin lesions, it took almost 100 years to realize that the disease is systemic and spares no organ and that it is caused by an aberrant autoimmune response. The clinical heterogeneity of the disease forced…
- December 1, 2011
- Tsokos G.C.
- N Engl J Med 2011; 365:2110-2121
The author reviews environmental, genetic, epigenetic, and hormonal factors in systemic lupus erythematosus, its diverse organ manifestations, and the myriad immune abnormalities that characterize this autodestructive disease, which mainly affects women of childbearing age.
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Original Article
Anti-Inhibitor Coagulant Complex Prophylaxis in Hemophilia with Inhibitors
After exposure to factor VIII, alloantibodies (inhibitors) that neutralize factor VIII clotting function develop in approximately 30% of patients with severe hemophilia A. The development of high-titer factor VIII inhibitors (>5 Bethesda units [BU]) complicates treatment because bleeding no longer…
- November 3, 2011
- Leissinger C., Gringeri A., Antmen B., et al.
- N Engl J Med 2011; 365:1684-1692
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- CME
In patients with hemophilia A in whom inhibitors of factor VIII develop, the prophylactic use of a factor VIII bypassing agent (anti-inhibitor coagulant complex) three times a week significantly reduced the risk of bleeding.
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Original Article
Brief Report: Inflammatory Skin and Bowel Disease Linked to ADAM17 Deletion
Inflammatory disorders of the skin and gut, including eczema, psoriasis, and celiac disease, have been linked to changes in barrier function and immune responses, by means of genetic and functional studies. Large case–control studies combined with genomewide association studies have identified…
- October 20, 2011
- Blaydon D.C., Biancheri P., Di W.-L., et al.
- N Engl J Med 2011; 365:1502-1508
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A mutation that abrogates the function of ADAM17, an enzyme that cleaves cell-surface proteins such as tumor necrosis factor α, was found to be associated with inflammatory skin and bowel disease in a pair of siblings.
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Interactive Medical Case
A Bird's-Eye View of Fever
A 78-year-old man presented to his primary care physician with a 4-month history of worsening fatigue, generalized weakness, and anorexia, with an unintentional weight loss of 11.4 kg (25 lb). He reported subjective fevers, chills, drenching night sweats, dry mouth, a nonproductive cough, dyspnea…
- October 13, 2011
- Ross J.J., Koo S., Lee A.I., Mushlin S.B., Milner D.A.
- N Engl J Med 2011; 365:e33
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- CME
A 78-year-old man presented with four months of worsening fatigue, generalized weakness, and anorexia with an unintentional weight loss of 25 pounds (11.4 kg). He reported subjective fevers, chills, drenching night sweats, dry mouth, nonproductive cough, ...
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Case Records of the Massachusetts General Hospital
Case 30-2011 — A 62-Year-Old Woman with Renal Failure
Presentation of Case. Dr. Kyle Staller (Medicine): A 62-year-old woman was admitted to this hospital because of renal failure. The patient had an autoimmune overlap syndrome with polymyositis, treated with prednisone and mycophenolate mofetil, but had been in her usual health until 6 weeks before…
- September 29, 2011
- Cunningham J., Harisinghani M.G., Taheri D.
- N Engl J Med 2011; 365:1233-1243
- CME
A 62-year-old woman with autoimmune overlap syndrome and polymyositis presented to this hospital with anemia, thrombocytopenia, and acute renal failure. Four days before admission, she was found on the floor of her home, confused and minimally conversant.
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Images in Clinical Medicine
Scleroderma
Figure 1.
- September 1, 2011
- Fernandes das Neves M. and Oliveira S.
- N Engl J Med 2011; 365:842
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A 75-year-old woman was evaluated for a history of inflammatory joint pain in both hands. Symptoms of gastroesophageal reflux were present. Examination revealed multiple palpable masses in the soft tissue overlying the extensor surfaces of the elbows, wrists, and hands.
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Clinical Implications of Basic Research
Systemic Lupus Erythematosus and the Neutrophil
Most physicians can identify B lymphocytes and T lymphocytes, and perhaps dendritic cells, as the cells involved in the pathogenesis of systemic lupus erythematosus (SLE). This systemic autoimmune disease is characterized by the loss of tolerance to nuclear antigens, the deposition of immune…
- August 25, 2011
- Bosch X.
- N Engl J Med 2011; 365:758-760
Recent studies implicate a peculiar form of neutrophil death and its extracellular sequelae in mediating inflammation in patients with systemic lupus erythematosus.
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Editorial
Hamartoma Syndromes, Exome Sequencing, and a Protean Puzzle
Gross malformations have been well known for centuries, even millennia. However, it was not until 1904 that Albrecht coined the concept of hamartoma. In 1934, the concept entered the field of developmental pathology generally in reference to tissue malformations. Hamartomata are localized…
- August 18, 2011
- Opitz J.M. and Jorde L.B.
- N Engl J Med 2011; 365:661-663
Gross malformations have been well known for centuries, even millennia. However, it was not until 1904 that Albrecht coined the concept of hamartoma. In 1934, the concept entered the field of developmental pathology generally in reference to tissue ...
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Original Article
A Mosaic Activating Mutation in AKT1 Associated with the Proteus Syndrome
The Proteus syndrome is characterized by patchy or segmental overgrowth and hyperplasia of multiple tissues and organs, along with susceptibility to the development of tumors, (Figure 1). It is thought that Joseph Merrick, an Englishman who lived in the late 19th century and became the subject of…
- August 18, 2011
- Lindhurst M.J., Sapp J.C., Teer J.K., et al.
- N Engl J Med 2011; 365:611-619
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The Proteus syndrome affects some tissues and not others and is thought to be caused by a somatic mutation. Investigators found that the mutation is caused by activation of AKT1, an enzyme that mediates glucose metabolism, cell proliferation, and apoptosis.
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Original Article
Wnt Signaling and Dupuytren's Disease
Dupuytren's disease is a benign fibromatosis of the hands and fingers, giving rise to the formation of nodules and cords and often leading to flexion contractures (Fig. 1 in the Supplementary Appendix, available with the full text of this article at NEJM.org). The prevalence of Dupuytren's disease…
- July 28, 2011
- Dolmans G.H., Werker P.M., Hennies H.C., et al.
- N Engl J Med 2011; 365:307-317
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Little is known about the cause of Dupuytren's disease, in which fibromatosis leads to flexion contractures of the hands and fingers. This genomic study suggests that one cause might be aberrations in the Wnt-signaling pathway.
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Clinical Problem-Solving
A Sleeping Giant
Foreword. In this Journal feature, information about a real patient is presented in stages (boldface type) to an expert clinician, who responds to the information, sharing his or her reasoning with the reader (regular type). The authors' commentary follows. Stage. A 71-year-old woman presented to…
- July 7, 2011
- Morris A., Grudberg S., Levy B.D., Loscalzo J.
- N Engl J Med 2011; 365:72-77
- CME
A 71-year-old woman presented with abdominal pain and night sweats. Four weeks earlier, headache, sinus pressure, and night sweats had developed, and amoxicillin was prescribed for presumed sinusitis. Her symptoms improved, but after 1 week, new abdominal pain developed.
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Images in Clinical Medicine
Knuckle Pads
Figure 1.
- June 23, 2011
- Nenoff P. and Woitek G.
- N Engl J Med 2011; 364:2451
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A healthy 10-year-old girl presented to her physician with nontender, well-circumscribed, mobile, slightly hyperkeratotic, erythematous plaques on the dorsal aspect of the interphalangeal joints of both thumbs. The plaques had developed over several months.
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Interactive Medical Case
A Sleeping Giant
A 71-year-old woman presented with abdominal pain and reported that intermittent drenching night sweats, a global nonpulsatile headache, sinus pressure, and left maxillary pain radiating into her teeth had developed 4 weeks earlier. Amoxicillin was prescribed for presumed sinusitis, and her…
- June 16, 2011
- Blair R.J., Ross J.J., Morris A., Grudberg S.
- N Engl J Med 2011; 364:e53
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- CME
A 71-year-old woman presented with abdominal pain and reported that intermittent drenching night sweats, a global nonpulsatile headache, sinus pressure, and left maxillary pain radiating into her teeth had developed 4 weeks earlier. Amoxicillin was ...
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Images in Clinical Medicine
Calcinosis Cutis in Systemic Sclerosis
Figure 1.
- June 9, 2011
- Makol A. and Ytterberg S.R.
- N Engl J Med 2011; 364:2245
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A 74-year-old man presented for evaluation of severe calcinosis cutis that had progressed to involve his fingers, hands, toes, right thorax, and right thigh, resulting in clinically significant functional limitation.
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Images in Clinical Medicine
Digital Gangrene
Figure 1.
- April 21, 2011
- Poisson J. and McCudden C.
- N Engl J Med 2011; 364:e34
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A 71-year-old man presented with palpable purpura and received a diagnosis of type II cryoglobulinemia. He had an elevated cryocrit (13%), evidence of a monoclonal IgM kappa immunoglobulin (0.8 g per deciliter), and a positive result for rheumatoid ...
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Case Records of the Massachusetts General Hospital
Case 11-2011 — A 47-Year-Old Man with Systemic Lupus Erythematosus and Heart Failure
Presentation of Case. Dr. Caroline L. Sokol (Medicine): A 47-year-old man with systemic lupus erythematosus (SLE) and progressive cardiac failure was transferred to this hospital after a cardiac arrest. One year before admission, dyspnea, leg edema, and abdominal distention gradually developed, and…
- April 14, 2011
- Newton-Cheh C., Lin A.E., Baggish A.L., Wang H.
- N Engl J Med 2011; 364:1450-1460
- Video
A 47-year-old man with systemic lupus erythematosus (SLE) was transferred to the hospital after cardiac arrest. Electrocardiography showed ST elevation, a long QT interval, and left ventricular hypertrophy (LVH). Echocardiography showed LVH and mitral-valve regurgitation.
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Editorial
The Burden of Functional Recovery from ARDS
The acute respiratory distress syndrome (ARDS) is commonly a cause for admission to the intensive care unit (ICU) and for mechanical ventilation. Within the past decade, research has shown not only that the need for acute supportive care is extensive and protracted, but also that the arc of…
- April 7, 2011
- Hall J.B. and Kress J.P.
- N Engl J Med 2011; 364:1358-1359
The acute respiratory distress syndrome (ARDS) is commonly a cause for admission to the intensive care unit (ICU) and for mechanical ventilation. Within the past decade, research has shown not only that the need for acute supportive care is extensive and ...
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Medical Meetings Pediatrics Conferences and Meetings

2012 Certifying Examinations of the American Board of Pediatrics

The general pediatrics examination will be held in various cities, Oct. 16-18. Registration for first-time applicants is ongoing through May 3. Registration for re-registrants is ongoing through May 24. The following subspecialty examinations will be held in various cities: "Hospice and Palliative Medicine" (Oct. 4); "Pediatric Transplant Hepatology" (Oct. 11); "Pediatric Cardiology" (Nov. 7); "Pediatric Pulmonology" (Nov. 8); "Medical Toxicology" (Nov. 12); and "Pediatric Critical Care Medicine" (Nov. 14). Registration for first-time applicants is ongoing through April 30. Registration for re-registrants is ongoing through June 15.

Contact the American Board of Pediatrics, 111 Silver Cedar Court, Chapel Hill, NC 27514-1513; or call (919) 929-0461; or fax (919) 918-7114 or (919) 929-9255; or see http://www.abp.org .

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Browse Rheumatology General

Showing 1 to 20 of 470 Articles

Filter Results

Lingual Raynaud's Phenomenon

Mechanisms of Disease: IgG4-Related Disease

Tophaceous Gout

Mechanisms of Disease: Systemic Lupus Erythematosus

Anti-Inhibitor Coagulant Complex Prophylaxis in Hemophilia with Inhibitors

Brief Report: Inflammatory Skin and Bowel Disease Linked to ADAM17 Deletion

A Bird's-Eye View of Fever

Case 30-2011 — A 62-Year-Old Woman with Renal Failure

Scleroderma

Systemic Lupus Erythematosus and the Neutrophil

Hamartoma Syndromes, Exome Sequencing, and a Protean Puzzle

A Mosaic Activating Mutation in AKT1 Associated with the Proteus Syndrome

Wnt Signaling and Dupuytren's Disease

A Sleeping Giant

Knuckle Pads

A Sleeping Giant

Calcinosis Cutis in Systemic Sclerosis

Digital Gangrene

Case 11-2011 — A 47-Year-Old Man with Systemic Lupus Erythematosus and Heart Failure

The Burden of Functional Recovery from ARDS

Medical Meetings Pediatrics Conferences and Meetings

2012 Certifying Examinations of the American Board of Pediatrics

Trends: Most Viewed (Last Week)