lupus,

If you have lupus, your immune system attacks healthy cells and tissues by mistake. This can damage your joints, skin, blood vessels and organs. There are many kinds of lupus. The most common type, systemic lupus erythematosus, affects many parts of the body. Discoid lupus causes a rash that doesn't go away. Subacute cutaneous lupus causes sores after being out in the sun. Another type can be caused by medication. Neonatal lupus, which is rare, affects newborns.
Anyone can get lupus, but women are most at risk. Lupus is also more common in African American, Hispanic, Asian and Native American women. The cause of lupus is not known.
Lupus has many symptoms. Some common ones are

• Joint pain or swelling
• Muscle pain
• Fever with no known cause
• Red rashes, often on the face (also called the "butterfly rash")

There is no one test to diagnose lupus, and it may take months or years to make the diagnosis. There is no cure for lupus, but medicines and lifestyle changes can help control it.

Tenosynovitis

Tenosynovitis

 involves inflammation of the tendon and tendon sheath. Examples of tenosynovitis include de Quervain tenosynovitis of the wrist (ie, abductor pollicis longus and extensor pollicis brevis tendons), volar flexor tenosynovitis (ie, trigger finger), pyogenic flexor tenosynovitis, which can be from gonococcal infections and other infectious etiologies.

Pathophysiology

Flexor tendons of the hand run in tight fibroosseous tunnels. Visceral and parietal layers of synovium lubricate and nourish the tendons. These layers are usually collapsed unless infection, which follows the path of least resistance along the tendon sheaths or inflammation, is present.
Infection can be introduced directly into the tendon sheaths through a skin wound (most often) or via hematogenous spread, as occurs with gonococcal tenosynovitis. Gonococcal infection originates as a mucosal infection of the genital tract, rectum, or pharynx. Dissemination occurs in approximately 1-3% of patients with mucosal infection. Approximately two thirds of patients with disseminated gonococcal infection develop tenosynovitis.
A history of recent trauma to the involved area is not uncommon and is believed to be a predisposing factor for the development of pyogenic flexor tenosynovitis.¹ Overuse leads to inflammation in de Quervain tenosynovitis. Etiology of volar flexor tenosynovitis is unknown.

Mortality/Morbidity

One complication of infectious tenosynovitis is a loss of active range of motion. A less frequent complication of infectious tenosynovitis is digit amputation, which occurs most commonly in very advanced cases. Pang et al conducted a review of 75 patients with pyogenic flexor tenosynovitis and found that the following risk factors were associated with poorer outcomes: (1) age older than 45 years; (2) presence of diabetes mellitus, renal failure, or peripheral vascular disease; (3) ischemic changes at the time of presentation; (4) subcutaneous purulence; and (5) polymicrobial infection at the time of surgery.²

Clinical

History

• de Quervain tenosynovitis
  • Patients have a history of repetitive pinching motion of the thumb and fingers (eg, assembly line work, driving in screws, weeding).
  • Pain in the radial aspect of the wrist becomes worse with activity and better with rest. Onset of pain is typically gradual in nature with no history of acute trauma. The affected area is shown in the image below.
  • 
    

    

    The first dorsal compartment of the wrist includes the tendon sheath that encloses the abductor pollicis longus and the extensor pollicis brevis tendons at the lateral border of the anatomic snuffbox.

    

    The first dorsal compartment of the wrist includes the tendon sheath that encloses the abductor pollicis longus and the extensor pollicis brevis tendons at the lateral border of the anatomic snuffbox.

  • Most common in middle-aged women
• Volar flexor tenosynovitis (ie, trigger finger)
  • This type of tenosynovitis most commonly affects the thumb or ring finger.
  • Most common in middle-aged women
  • More common in patients with diabetes
  • Locking of involved finger in flexion is followed by sudden release (hence the name trigger finger); hand pain radiates to fingers. In more severe cases, the finger may require passive manipulation to regain extension.
• Gonococcal tenosynovitis
  • This type of inflammation most commonly affects teenagers and young adults; gonococcal tenosynovitis is more common in women, especially during pregnancy or after menstruation, when dissemination of gonorrhea is more likely to occur.
  • Interval from sexual exposure to onset of symptoms of dissemination can vary from 1 day to several weeks.
  • Vaginal or penile discharges are usually absent; fever, chills, malaise, and polyarthralgias are common.
  • Most common sites affected are the dorsum of the wrist, hand, and ankle.
• Nongonococcal infectious tenosynovitis
  • A puncture wound, dry cracked skin, laceration, bite, or high-pressure injection injury (eg, paint, grease gun) may be present.
  • Frequently, no obvious portal of injury is present.
  • Pain and swelling occur along affected tendon; flexor hand tendons are most commonly involved.

Physical

• de Quervain tenosynovitis
  • Pain occurs on palpation along the radial aspect of the wrist.
  • Pain occurs with passive range of motion of the thumb.
  • Pain occurs with ulnar deviation of the wrist with the thumb cupped in a closed fist; this is termed the Finkelstein test and is shown in the image below.
  • 
    

    

    The Finkelstein test is performed by having the patient make a fist with the thumb inside the fingers. The clinician then applies ulnar deviation of the wrist to reproduce the presenting symptoms of dorsolateral wrist pain.

    

    The Finkelstein test is performed by having the patient make a fist with the thumb inside the fingers. The clinician then applies ulnar deviation of the wrist to reproduce the presenting symptoms of dorsolateral wrist pain.

• Volar flexor tenosynovitis (ie, trigger finger)
  • Tenderness is present at the proximal end of the tendon sheath, in the distal palm (just proximal to metacarpal head).
  • Palpable tendon thickening and nodularity may be present.
  • Crepitation and catching of the tendon may be appreciated when the finger is flexed.
• Gonococcal tenosynovitis
  • Erythema, tenderness to palpation, and painful range of motion of the involved tendon(s) are present.
  • Fever is common.
  • Dermatitis is also common (occurs in approximately two thirds of disseminated gonococcal) and is characterized by hemorrhagic macules or papules on the distal extremities or trunk.
• Nongonococcal infectious tenosynovitis
  • Tenderness, erythema, and painful range of motion of the involved tendon(s) are present.
  • Cardinal signs of Kanavel include the following: (1) fusiform swelling of the finger (swelling along the whole digit), (2) flexed position of the finger, (3) severe pain with passive extension of the finger, and (4) tenderness and swelling along and limited to the flexor tendon sheath.

Causes

• de Quervain tenosynovitis: Overuse leads to thickening of the extensor retinaculum of the first dorsal compartment and narrowing of the fibroosseous canal.
• Volar flexor tenosynovitis (ie, trigger finger): Overuse is thought to be the most common cause, but multiple etiologies have been identified. The triggering phenomenon is thought to be caused by hypertrophy of the first annular pulley.
• Gonococcal tenosynovitis: Neisseria gonorrhoeae is a cause.
• Nongonococcal infectious tenosynovitis
  • Staphylococcus aureus and Streptococcus species are the most common etiologic agents, but infection is frequently mixed (aerobic and anaerobic).
  • Pasteurella multocida is common with cat bites; Eikenella corrodens occurs with human bites. However, human and animal bites may have a mixture of aerobic and anaerobic flora.
  • Predisposing factors include diabetes mellitus, intravenous (IV) drug abuse, debility, and arteriosclerosis obliterans.
  • Mycobacterium species can also cause tenosynovitis, particularly in immunocompromised patients.

Rheumatic fever

The incidence of acute rheumatic fever (ARF) has declined in most developed countries, and many physicians have little or no practical experience with the diagnosis and management of this condition. Occasional outbreaks in the United States make complacency a threat to public health.
Diagnosis rests on a combination of clinical manifestations that can develop in relation to group A streptococcal pharyngitis. These include chorea, carditis, subcutaneous nodules, erythema marginatum, and migratory polyarthritis. Because the inciting infection is completely treatable, attention has been refocused on prevention.

Pathophysiology

Although the inciting bacterial agent is well known, susceptibility factors remain unclear. The location of the streptococcal infection seems to play an important role. The clinical syndrome typically follows a streptococcal pharyngitis, but streptococcal cellulitis has never been implicated.
The earliest and most common feature is a painful migratory arthritis, which is present in approximately 80% of patients. Large joints such as knees, ankles, elbows, or shoulders are typically affected. Sydenham chorea was once a common late-onset clinical manifestation but is now rare. Carditis (with progressive congestive heart failure, a new murmur, or pericarditis) may be the presenting sign of unrecognized past episodes and is the most lethal manifestation.
Genetics may contribute, as evidenced by an increase in family incidence. No significant association with class-I human leukocyte antigens (HLAs) has been found, but an increase in class-II HLA antigens DR2 and DR4 has been found in black and white patients, respectively. Evidence suggests that elevated immune-complex levels in blood samples from patients with ARF are associated with HLA-B5.¹

Frequency

United States

The incidence of an acute rheumatic episode following streptococcal pharyngitis is 0.5-3%. The peak age is 6-20 years. Although the incidence of ARF has steadily declined, the mortality rate has declined even more steeply. Credit can be attributed to improved sanitation and antibiotic therapy. Several sporadic outbreaks in the United States could not be blamed directly on poor living conditions. New virulent strains are the best explanation.

International

Most major outbreaks occur under conditions of impoverished overcrowding where access to antibiotics is limited. Rheumatic heart disease accounts for 25-50% of all cardiac admissions internationally. Regions of major public health concern include the Middle East, the Indian subcontinent, and some areas of Africa and South America. As many as 20 million new cases occur each year. The introduction of antibiotics has been associated with a rapid worldwide decline in the incidence of ARF. Now, the incidence is 0.23-1.88 patients per 100,000 population. From 1862-1962, the incidence declined from 250 patients to 100 patients per 100,000 population, primarily in teenagers. Notably, natives of Polynesian ancestry in Hawaiian and Maori populations are an exception. The incidence continues to be 13.4 patients per 100,000 hospitalized children per year.²

Mortality/Morbidity

• Mortality rates are steadily improving because of better sanitation and health care.
• The current pattern of morbidity is difficult to measure because the first attack of rheumatic fever follows an unpredictable course. As many as 90% of episodes are clinically contained within 3 months.
• Carditis causes the most severe clinical manifestation because heart valves can be permanently damaged. The disorder also can involve the pericardium, myocardium, and the free borders of valve cusps. Death or total disability may occur years after the initial presentation of carditis.

Race

• An association between certain class-II HLA antigens (DR2 in blacks and DR4 in whites) and ARF has been reported.

Sex

• No general clear-cut sex predilection for the syndrome has been reported, but its manifestations seem to be sex variable. For example, certain clinical manifestations (ie, chorea and tight mitral stenosis) are predominant in women, while men are more likely to develop aortic stenosis.

Age

• The initial attack of ARF occurs most frequently in persons aged 6-20 years and rarely occurs in persons older than 30 years.
• The disease may cluster in families.
• In some countries, a shift into older groups may be a trend.

Clinical

History

• Diagnosis is challenging for several reasons, as follows:
  • Approximately 70% of older children and young adults recollect pharyngitis. However, only approximately 20% of young children recollect pharyngitis. Therefore, younger children who present with signs or symptoms consistent with acute rheumatic fever (ARF) merit a higher index of suspicion.
  • The rate of isolation of group A streptococci from the oropharynx is extremely low in all populations.
• Usually, a latent period of approximately 18 days occurs between the onset of streptococcal pharyngitis and ARF. This latent period is rarely shorter than 1 week or longer than 5 weeks.
  • Typically, the first manifestation is a very painful migratory polyarthritis. Often, associated fever and constitutional toxicity develop.
  • Acute attacks usually resolve within 12 weeks.
• Guidelines for diagnosis published more than 50 years ago by T. Duckett Jones³ have been slightly revised by the American Heart Association (AHA).⁴ Prior history of a preceding group A streptococcal infection is helpful but not required. In addition, 2 major manifestations or 1 major and 2 minor manifestations must be present.
  • Major manifestations include carditis, polyarthritis, chorea, erythema marginatum, and subcutaneous nodules.
  • Minor manifestations include arthralgias and fever. Laboratory findings include elevated levels of acute-phase reactants (erythrocyte sedimentation rate [ESR] and C-reactive protein) and a prolonged PR interval. A prolonged PR interval is not specific and has not been associated with later cardiac sequelae. The utility of echocardiography is also controversial.
• The Jones criteria⁴ should be viewed as a guide to determine who is at high risk but cannot be used to define diagnosis with absolute certainty.
  • An exception includes chorea, which can present as the sole manifestation of ARF, in spite of negative laboratory results.
  • Another possible exception is indolent carditis.
• A throat culture with results positive for Streptococcus is found in approximately 25% of patients at the time of presentation.

Physical

• Physical findings can be nonspecific and misleading; therefore, a high index of suspicion is required for diagnosis.
• Suspicious signs for carditis include new or changing valvular murmurs, cardiomegaly, congestive heart failure, and/or pericarditis.
• Nearly 60% of patients with carditis develop isolated mitral valve involvement, followed in prevalence by combined mitral and aortic valve involvement.
• When present, Sydenham chorea is seldom evident at the time of initial presentation.
• Erythema marginatum and subcutaneous nodules are rare (<10% of patients).
  
  

  

  Erythema marginatum, the characteristic rash of acute rheumatic fever.

  

  Erythema marginatum, the characteristic rash of acute rheumatic fever.

• Arthritis, which occurs in 80% of patients, usually involves multiple large joints, particularly the knees, ankles, elbows, and wrists.
  • Hips and smaller joints of hands and feet are less commonly involved.
  • Migratory polyarthritis is usually associated with a febrile illness. It involves a series of painful joints, followed by another series of painful joints.
  • This form of arthritis rarely causes permanent joint deformity.
• Unusual presentations, such as indolent carditis and isolated chorea, may also occur. Even rarer manifestations include epistaxis and abdominal pain due to serositis.

Causes

• Although the mechanism by which streptococcal organisms cause disease is not entirely clear, overwhelming epidemiologic evidence suggests that ARF is caused by streptococcal infection, and recurrences can be prevented with prophylaxis.
• Strains of group A streptococci that are heavily encapsulated and rich in M protein (signifying virulence in streptococcal strains) seem to be most likely to result in infection.
• Group A Streptococcus is thought to cause the myriad of clinical diseases in which the host's immunologic response to bacterial antigens cross-react with various target organs in the body, resulting in molecular mimicry. In fact, autoantibodies reactive against the heart have been found in patients with rheumatic carditis. The antibody can cross-react with brain and cardiac antigens, and immune complexes are present in the serum. The problem has been the uncertainty of whether these antibodies are the cause or result of myocardial tissue injury.

Gonococcal arthritis

Gonococcal arthritis is caused by infection with the gram-negative diplococcus Neisseria gonorrhoeae. In the United States, gonococcal arthritis is the most common form of septic arthritis.¹ This is in contrast to Western Europe, where gonococcal arthritis is uncommon,² likely owing to a 70% decline in gonococcal infections over the last 2 decades.¹
Although the pathogenesis of articular involvement is controversial, it is ultimately a consequence of disseminated gonococcal infection (DGI). Gonococcal arthritis manifests as either a bacteremic infection (arthritis-dermatitis syndrome; 60% of cases) or as a localized septic arthritis (remaining 40%). Arthritis-dermatitis syndrome includes the classic triad of dermatitis, tenosynovitis, and migratory polyarthritis.
Patients with gonococcal arthritis usually require initial hospitalization for intravenous antibiotic therapy; upon improvement, they can be transitioned to oral antibiotics. Unlike in Staphylococcus aureus septic arthritis, joint destruction is rare in gonococcal arthritis.

Pathophysiology

N gonorrhoeae is a highly infectious organism capable of colonizing diverse mucosal surfaces. The risk of infection from a single contact with the organism is estimated at 60%-90% among women and 20%-50% among men.¹ Common sites of infection include the urethra, cervix, pharynx, and rectum; however, infection may be asymptomatic in some patients. Hematogenous spread of the mucosal infection occurs in 0.5%-3% of cases,³ and disseminated infection is thought to play a major role in the pathogenesis of gonococcal arthritis. Patients with DGI may present with dermatitis-arthritis syndrome or with a localized septic arthritis. These presentations may represent different phases of a disease continuum.
Factors that correlate with increased risk of a disseminated infection have been identified for both the host and the organism.
Host factors for disseminated infection include the following:²

• Female sex
• Pregnancy
• Menses
• Systemic lupus erythematosus
• Complement deficiency
• Low socioeconomic or educational status
• Intravenous drug use
• HIV infection
• Multiple sexual partners

Characteristics of the gonococcus associated with DGI include the following:^1,2,3

• Antigenic variation of pili
• Protein IA on the outer membrane (inhibits host factor H and C4-binding protein, making host complement cascade less effective)
• Lack of protein II
• AHU strains with nutritional requirements for arginine, hypoxanthine, and uracil (often associated with protein IA)

Frequency

United States

In 2005, 339,593 cases of gonococcal infection were reported in the United States, making it the second most commonly reported communicable disease.⁴ Although rates of gonococcal infection declined from 1975-1997, the national rate of gonococcal infection increased in 2005 to 115 cases per 100,000 persons.⁴ However, rates vary by region and demographics, as described below.

International

• According to the World Health Organization, gonococcal infection is among the curable sexually transmitted infections, of which 340 million cases occur annually.⁵
• The incidence of gonococcal infection is lower in Europe than in North America. For example, the incidence of gonococcal infection in Sweden in 1992 was less than 5 per 100,000 population, while the incidence in the United States in 1995 was 150 per 100,000.²
• Gonococcal infection is high in developing countries, partly because of limited public health infrastructure and limited access to health care.

Mortality/Morbidity

Morbidity associated with DGI has decreased dramatically in the postantibiotic era. Complications of DGI including pericarditis, endocarditis, meningitis, perihepatitis, pyomyositis, osteomyelitis, and glomerulonephritis are now rare and occur in only 1%-3% of cases.¹

Race

In the United States, gonococcal infection is most common in African Americans.⁴ The prevalences in with, Hispanic, Native American, and Asian populations are similar and dramatically lower than in African Americans.⁴

Sex

The disease is 3-4 times more common in females than in males, possibly because of the increased risk of asymptomatic infection in females.²

Age

The highest rates of infection in the United States are among persons aged 15-29 years; however, older adults may be affected.⁴

Clinical

History

The clinical presentation of disseminated gonococcal infection (DGI) is typically divided into a bacteremic form and a septic arthritis form. Approximately 60% of patients present with symptoms consistent with the bacteremic form, and the remaining 40% present with symptoms of more localized infection. Although each form presents with its own symptom complex, the overlap can be considerable. The time from initial infection to initial manifestations of DGI ranges from 1 day to 3 months.¹

• Bacteremic form (arthritis-dermatitis syndrome)⁶
  • Symptoms are typically present 3-5 days before diagnosis.
  • Migratory arthralgias are the most common presenting symptom in persons with DGI and are usually polyarticular. The arthralgias are typically asymmetric and tend to involve the upper extremities more than the lower extremities. The wrist, elbows, ankles, and knees are most commonly affected. Symptoms resolve spontaneously in 30%-40% of cases or evolve into a septic arthritis in one or several joints.
  • Pain may also be due to tenosynovitis. The tenosynovitis of DGI is asymmetric and most commonly occurs over the dorsum of the wrist and hand, as well as over the metacarpophalangeal joints, ankles, and knees. Diffuse involvement of fingers can result in dactylitis.¹
  • The rash associated with the bacteremic form of DGI may be overlooked by patients because it is painless and nonpruritic and consists of small papular, pustular, or vesicular lesions.
  • Nonspecific constitutional symptoms may include myalgias, fever, and malaise.
• Septic arthritis form⁶
  • Joint symptoms begin within days to weeks of gonococcal infection.
  • Patients may experience pain, redness, and swelling in usually one or sometimes multiple joints, most commonly the knees, wrists, ankles, and elbows.¹

Physical

• Bacteremic form (classic triad of migratory polyarthritis, tenosynovitis, and dermatitis)⁶
  • Migratory arthritis has an asymmetric distribution, most commonly affecting wrists, ankles, and elbows. Seventy percent of patients have 1-3 joints with clear inflammatory signs after just a few days. Symmetric polyarthritis is less common but may occur in approximately 10% of patients.
  • Tenosynovitis is asymmetric, usually affecting the dorsum of wrists, hands, and ankles. Tenosynovitis of the fingers may result in dactylitis.
  • Dermatitis occurs in 40%-70% of patients and typically involves the extremities. Lesions are usually tiny maculopapular, pustular, or vesicular lesions on an erythematous base. The center of the lesion may become necrotic or hemorrhagic. Despite their appearance, they are painless and nonpruritic. The lesions tend to disappear within a few days after treatment is initiated. Usually, 4-50 lesions are reported. Rarely, the lesions may resemble erythema nodosum or erythema multiforme.
  • Fever rarely involves a temperature of greater than 39°C.
  • Other presentations of DGI include the following, which now occur in only 1%-3% of cases:¹
    • Fitz-Hugh-Curtis syndrome (gonococcal perihepatitis)
    • Sepsis with Waterhouse-Friderichsen syndrome
    • Gonococcal endocarditis (rare in the antibiotic era)
    • Gonococcal meningitis (very rare in the antibiotic era)
• Septic arthritis form⁶
  • Septic arthritis is characterized by acute arthritis with signs of joint effusion, warmth, tenderness, decreased range of motion, and marked erythema.
  • Septic arthritis most commonly involves the wrists, hands, knees, and elbows. Chronic arthritis with joint destruction is rare with appropriate antibiotic therapy.

Causes

Gonococcal arthritis is caused by infection with the gram-negative diplococcus N gonorrhoeae. The risk of dissemination following mucosal infection depends on both the ability of the patient's immune system to control the infection and the virulence of the organism. See Pathophysiology.