Calcifying Tendonitis

Dupuytren contracture results from contracture of the palmar fascia within the hand, possibly resulting in a fixed flexion deformity of the metacarpophalangeal (MCP) joints and the proximal interphalangeal (PIP) joints. This condition usually affects the fourth and fifth digits (the ring and small fingers). (See the images below.)
Arrow denotes the cord often present in Dupuytren contracture. Metacarpophalangeal joint and proximal interphalangeal joint contractures are also present. This photo shows a patient with an inability to extend the fourth and fifth digits. The differential diagnosis includes Dupuytren contracture, which is a flexion contracture most commonly involving digits 4 and/or 5. The condition is a fibrosing disorder that results in slowly progressive thickening and shortening of the palmar fascia, leading to the debilitating digital contractures. Dupuytren contracture belongs to the group of fibromatoses that include plantar fibromatosis (Ledderhose disease), penile fibromatosis (Peyronie disease), and fibromatosis of the dorsal proximal interphalangeal (PIP) joints (Garrod nodes or knuckle pads).^[1] Although many cases appear to be idiopathic and without coexisting conditions, a variety of associated diseases have been reported. (See Etiology.)
Dupuytren contracture is most commonly observed in persons of Northern European descent and affects 4-6% of whites worldwide.^[2] Many individuals have bilateral disease (45%); in unilateral cases, the right side is more often affected.^[3] The ring finger is most commonly involved, followed by the fifth digit and then the middle finger. The index finger and the thumb are typically spared. (See Epidemiology and Presentation.)
Although the cause of Dupuytren disease is unknown, a family history is often present. Males are 3 times as likely to develop disease and are more likely to have higher disease severity.^{[4, 5]} Male predominance may be related to expression of androgen receptors in Dupuytren fascia. (See Etiology and Epidemiology.)^[6]
Additional risk factors include manual labor with vibration exposure, prior hand trauma, alcoholism, smoking, diabetes mellitus, hyperlipidemia, Peyronie disease, and complex regional pain syndrome.^[7] Rheumatoid arthritis seems to protect against the development of Dupuytren disease. (See Etiology.)
Therapies include conservative medical and surgical modalities. Although the condition is not fatal, significant morbidity can occur if patients remain untreated. (See Prognosis, Treatment, and Medication.)

Stages of Dupuytren disease

Dupuytren disease occurs in the following 3 stages:

• Proliferative phase - Local fascial fibroplasia and development of a nodule, in which myofibroblasts proliferate, occur (see the image below), with palmar skin blanching on finger extension; in early disease, some patients may report tenderness and discomfort associated with the nodules
• Involutional phase - Contracture develops, with associated nodular thickening of the palmar fascia; myofibroblasts are predominant during this phase and align themselves along tension lines within the nodule
• Residual phase - The nodular tissue disappears, leaving acellular tissue and thick bands of collagen; the ratio of type III collagen to type I collagen increases, which is the reverse of the normal pattern in the palmar fascia^[8]

Grades of severity

The grading system for Dupuytren disease severity is as follows (see the images below)^[9] :

• Grade 1 - Thickened nodule and band in the palmar aponeurosis; may have associated skin abnormalities
• Grade 2 - Development of pretendinous and digital cords with limitation of finger extension
• Grade 3 - Presence of flexion contractureArrow denotes the cord often present in Dupuytren contracture. Metacarpophalangeal joint and proximal interphalangeal joint contractures are also present. Arrow denotes the typical cords of Dupuytren contracture. These cords are usually painless. Note the metacarpophalangeal joint contracture. This photo demonstrates the presence of a nodule as well as skin blanching with extension of the affected digits. Three clinical grades of Dupuytren disease.

Overview of Rheumatoid Spondylitis

The most common sites of rheumatoid arthritis (RA) are the metatarsophalangeal joints, followed by the metacarpophalangeal joints and the cervical spine (ankylosing spondylitis, rheumatoid spondylitis). Much of the understanding of spinal afflictions in rheumatoid arthritis was advanced by studies published in the 1950s and 1960s.^[1] In 1951, Davis and Markley detailed medullary compression as a cause of death in patients with rheumatoid arthritis.^[2] In 1969, Mathews reported that 25-30% of patients with rheumatoid arthritis who were admitted to the hospital had radiographic evidence of cervical spine involvement.^[3]
Rheumatoid spondylitis (ankylosing spondylitis) primarily affects the cervical spine. Affliction of the thoracic or lumbar spine is rare. The anatomic abnormalities occur as a consequence of the destruction of synovial joints, ligaments, and bone. Abnormalities of the rheumatoid cervical spine generally can be grouped into 3 categories that may be seen in isolation or combined involvement, of which atlantoaxial instability (AAI) or atlantoaxial subluxation (AAS) is the most common abnormality (see the image below). AAS can be a fixed deformity or can be partially or fully reducible.
Rheumatoid spondylitis. Depiction of anterior subluxation of C1 on C2, retrodental pannus, and osseous erosions; the spinal cord is compressed between the pannus anteriorly and the posterior arch of the atlas. Superior migration of the odontoid (SMO) is the next most common abnormality and has alternately been referred to as cranial settling, pseudobasilar invagination, or vertical/upward translocation of the odontoid (see the following images).^[4] The third and least common deformity is subaxial subluxation, which may be seen at multiple levels and produces a stepladder deformity.
Rheumatoid spondylitis. Depiction of superior migration of the odontoid into the foramen magnum with compression of the spinal cord. Rheumatoid spondylitis. Pertinent measurements of superior migration of the odontoid; cranial migration distance (CMD).

For patient education information, see eMedicine's Arthritis Center, as well as Rheumatoid Arthritis and Rheumatoid Arthritis Medications.

Go to Rheumatoid Arthritis and Ankylosing Spondylitis for more information on these topics.

Bursitis

Bursae are saclike structures between skin and bone or between tendons, ligaments, and bone. They are lined by synovial tissue, which produces fluid that lubricates and reduces friction between these structures. Bursitis occurs when the synovial lining becomes thickened and produces excessive fluid, leading to localized swelling and pain.^{[1, 2, 3]}
Olecranon bursitis, shown here with the elbow flexed. Image courtesy of UMDNJ-New Jersey Medical School, www.DoctorFoye.com, and www.TailboneDoctor.com. Olecranon bursitis aspiration of a hemorrhagic effusion. Image courtesy of UMDNJ-New Jersey Medical School, www.DoctorFoye.com, and www.TailboneDoctor.com. Location of pes anserine bursa on the medial knee. MCL is medial collateral ligament.

Recent studies

In a study of 25 cases of postarthroplasty trochanteric bursitis requiring corticosteroid injection, Farmer et al found that corticosteroid injections were effective treatment and that nonoperative management may be more likely to fail in young patients and patients with leg-length discrepancies. Of the 25 hips, 11 required multiple corticosteroid injections, and symptoms resolved in 20 but not in 5 cases.^[4]
Pretell et al described distal "Z" lengthening of the fascia lata in 13 hips and reported that 12 of the 13 patients reported good results. According to the authors, this technique is less aggressive and can be performed under local anesthesia with little morbidity and disability. Mean surgical time for the procedure was 15 minutes, and 1 seroma was reported as a complication.^[5]
According to Martinez-Taboada et al, in patients with severe septic bursitis, but without extensive cellulitis, aspiration plus I.V. cloxacillin may be sufficient treatment, and in patients with more severe cases of septic bursitis, aspiration along with cloxacillin plus gentamicin may be appropriate in the majority. The investigators studied 82 patients with severe septic bursitis, and the most frequent bacterium isolated was Staphylococcus aureus

Scleroderma

Scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin). Hippocrates first described this condition as thickened skin.^[1] Carlo Curzio (1752) offered the first detailed description of scleroderma in a patient with hard skin, which he described as woodlike or as containing a dry hide. In 1836, Giovambattista Fantonetti applied the term scleroderma to a patient's condition.^[2] He applied the term to describe a patient with dark leatherlike skin who exhibited a loss of range of joint motion due to skin tightening. Robert H. Goetz first described in detail the concept of scleroderma as a systemic disease in 1945; he introduced the term progressivesystemic sclerosis  to emphasize the systemic and often progressive nature of the disease.

Definition

The term systemic sclerosis is used to describe a systemic disease characterized by skin induration and thickening accompanied by various degrees of tissue fibrosis and chronic inflammatory infiltration in numerous visceral organs, prominent fibroproliferative vasculopathy, and humoral and cellular immune alterations.
The American College of Rheumatology (ACR) criteria for the classification of systemic sclerosis require one major criterion or two minor criteria, as follows:

• Major criterion: Proximal scleroderma is characterized by symmetric thickening, tightening, and induration of the skin of the fingers and the skin that is proximal to the metacarpophalangeal or metatarsophalangeal joints. These changes may affect the entire extremity, face, neck, and trunk (thorax and abdomen; see images below). Tightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles. Sclerodactyly with digital ulceration, loss of skin creases, joint contractures, and sparse hair.
• Minor criteria

  • Sclerodactyly is characterized by thickening, induration, and tightening of the skin, limited to only the fingers.
  • Digital pitting scars or a loss of substance from the finger pad: As a result of ischemia, depressed areas of the fingertips or a loss of digital pad tissue occurs.
  • Bibasilar pulmonary fibrosis includes a bilateral reticular pattern of linear or lineonodular densities most pronounced in basilar portions of the lungs on standard chest roentgenography. These densities may assume the appearance of diffuse mottling or a honeycomb lung and are not attributable to primary lung disea

Sjogren Syndrome

Sjögren syndrome is a systemic chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine organs. Most individuals with Sjögren syndrome present with sicca symptoms, such as xerophthalmia (dry eyes), xerostomia (dry mouth), and parotid gland enlargement, which is seen in the image below. (See Presentation.)
Marked bilateral parotid gland enlargement in a patient with primary Sjögren syndrome. Sicca syndrome is a common clinical finding. In addition, numerous extraglandular features may develop, such as arthralgia, arthritis, Raynaud phenomenon, myalgia, pulmonary disease, gastrointestinal disease, leukopenia, anemia, lymphadenopathy, neuropathy, vasculitis, renal tubular acidosis, and lymphoma. About 50% of patients with Sjögren syndrome have cutaneous findings, such as dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria.^[1] (See Etiology, Presentation, and Workup.)
Primary Sjögren syndrome occurs in the absence of another underlying rheumatic disorder, whereas secondary Sjögren syndrome is associated with another underlying rheumatic disease, such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), or scleroderma. Given the overlap of Sjögren syndrome with many other rheumatic disorders, it is sometimes difficult to determine whether a clinical manifestation is solely a consequence of Sjögren syndrome or is due to one of its overlapping disorders.
Importantly, classic clinical features of Sjögren syndrome may also be seen in viral infections with hepatitis C, human immunodeficiency virus (HIV), and human T-cell lymphotrophic virus (HTLV). Treatment for Sjögren syndrome is largely based on symptoms, but patients must be monitored carefully for the potential development of lymphoma. (See DDx.)

American-European Consensus Group classification

A number of classification criteria for Sjögren syndrome were designed primarily for clinical research studies but are also often used to help guide clinical diagnoses. The American-European Consensus Group’s criteria for the classification of Sjögren syndrome are outlined below.^{[2, 3]} These are the most currently accepted criteria for the diagnosis of Sjögren syndrome. These criteria allow a diagnosis of Sjögren syndrome in patients without sicca symptoms or who have not undergone a biopsy.
According to the American-European classification system (as modified by Tzioufas and Voulgarelis^[4] ), diagnosis of primary Sjögren syndrome requires 4 of 6 of the below criteria; in addition, either criterion number 5 or criterion number 6 must be included. Sjögren syndrome can be diagnosed in patients who have no sicca symptoms if 3 of 4 objective criteria are fulfilled. The criteria are as follows:

• Ocular symptoms - Dry eyes for more than 3 months, foreign-body sensation, use of tear substitutes more than 3 times daily
• Oral symptoms - Feeling of dry mouth, recurrently swollen salivary glands, frequent use of liquids to aid swallowing
• Ocular signs - Schirmer test performed without anesthesia (< 5 mm in 5 min), positive vital dye staining results
• Oral signs - Abnormal salivary scintigraphy findings, abnormal parotid sialography findings, abnormal sialometry findings (unstimulated salivary flow < 1.5mL in 15min)
• Positive minor salivary gland biopsy findings
• Positive anti–SSA or anti–SSB antibody results

Secondary Sjögren syndrome is diagnosed when, in the presence of a connective-tissue disease, symptoms of oral or ocular dryness exist in addition to criterion 3, 4, or 5, above.
Application of these criteria has yielded a sensitivity of 97.2% and a specificity of 48.6% for the diagnosis of primary Sjögren syndrome. For secondary Sjögren syndrome, the specificity is 97.2% and the sensitivity, 64.7%.^[5]
Exclusion criteria include past head-and-neck irradiation, hepatitis C infection, acquired immunodeficiency syndrome (AIDS), prior lymphoma, sarcoidosis, graft versus host disease, and the use of anticholinergic drugs.

Complications

Complications related to Sjögren syndrome include the following (see Prognosis, Treatment, and Medication):

• Emergence of disorders associated with Sjögren syndrome, such as SLE and RA
• Infection of the parotid gland, typically staphylococcal, streptococcal, or pneumococcal - Clues include unilateral worsening of symptoms, along with tenderness, warmth, and erythema
• Emergence of parotid tumors - Watch for unusually hard or unilateral parotid enlargement
• Pregnant patients with antiRo/SS-A antidodies are at risk for fetal loss, complete heart block in the fetus ,and neonatal lupus syndrome in the newborn
• Emergence of pseudolymphomas (pleomorphic cells that do not meet the criteria for malignancy) andnon-Hodgkin B-cell lymphomas (see the image below)^[4] Clinical photograph and photomicrograph of a 48-year-old man with Sjögren syndrome with a large left parotid mass. On biopsy, B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type was identified. Microscopic section of parotid biopsy, stained with immunoperoxidase for kappa light chains (brown-stained cells), showed monoclonal population of B cells, confirming the diagnosis.

Leukocytoclastic Vasculitis

Leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis and hypersensitivity angiitis, is a histopathologic term commonly used to denote a small-vessel vasculitis (see image shown below). Leukocytoclastic vasculitis has many causes, but no cause is identified in up to 50% of patients with this condition.
Histopathology of leukocytoclastic vasculitis. Leukocytoclastic vasculitis may be localized to the skin or may manifest in other organs. The internal organs affected most commonly include the joints, the gastrointestinal tract, and the kidneys. The prognosis is good in the absence of internal involvement.
Leukocytoclastic vasculitis may be acute or chronic. Chronic disease that primarily involves the skin should be treated with nontoxic modalities whenever possible, avoiding the use of systemic corticosteroids and immunosuppressive agents.
For additional information on cutaneous manifestations of leukocytoclastic vasculitis, see the eMedicine articleHypersensitivity Vasculitis (Leukocytoclastic Vasculitis)  in the Dermatology volume.

Polyarteritis Nodosa

Classicpolyarteritis nodosa (PAN or c-PAN) is a systemic vasculitis characterized by necrotizing inflammatory lesions that affect medium-sized and small muscular arteries, preferentially at vessel bifurcations, resulting in microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction.
Kussmaul and Maier first described PAN in 1866. The autopsy of a patient with fever, weight loss, abdominal pain, and polyneuropathy revealed areas of focal inflammatory exudations that gave rise to palpable nodules along the course of medium-sized arteries.^[1] PAN, like other vasculitides, affects multiple systems and has protean manifestations, although it most commonly affects skin, joints, peripheral nerves, the gut, and the kidney.^[2] The lungs are usually spared with PAN. A typical PAN patient might present with fever, night sweats, weight loss, skin ulcerations or tender nodules, and severe muscle and joint pains developing over weeks or months. (See Etiology, Clinical, and Workup.)
Nonspecific, firm, tender subcutaneous nodules without livedo reticularis and/or systemic involvement may be the first sign of polyarteritis nodosa (PAN). Insight into PAN requires some understanding of how this rare disease has been defined. Periarteritis nodosa was a term used from the mid 1800s to the 1900s to describe a spectrum of systemic vasculitic disorders, including diseases that manifested as arterial aneurysms, as well as those that caused diffuse necrotizing glomerulonephritis.^{[3, 4]} The term periarteritis nodosa was changed to polyarteritis nodosa in the mid 1900s to reflect the transmural inflammation of arteries caused by this disorder.^[5]
The understanding of vasculitides continued to increase by the 1980s with the discovery of antineutrophil cytoplasmic antibodies (ANCAs). Microscopic polyangiitis
 (MPA; formerly called microscopic polyarteritis) is an ANCA-associated systemic vasculitis that has some features similar to those of classic PAN, with the additional involvement of renal glomeruli and pulmonary capillaries.

Features of PAN

The American College of Rheumatology (ACR) established criteria for research purposes in order to differentiate PAN from other forms of vasculitis.^[6] A committee of ACR physicians selected 10 disease features of PAN; in order for PAN to be diagnosed, at least 3 of the 10 ACR criteria should be present when radiographic or pathological diagnosis of vasculitis is made^[6] (See Clinical and Workup.):

• Weight loss of 4 kg or more
• Livedo reticularis
• Testicular pain/tenderness
• Myalgia or leg weakness/tenderness
• Mononeuropathy or polyneuropathy
• Diastolic blood pressure greater than 90 mm/Hg
• Elevated blood urea nitrogen (BUN) or creatinine level unrelated to dehydration or obstruction
• Presence of hepatitis B surface antigen or antibody in serum
• Arteriogram demonstrating aneurysms or occlusions of the visceral arteries
• Biopsy of small- or medium-sized artery containing polymorphonuclear neutrophils

The strong association of MPA with ANCA, as well as the pathologic and clinical differences between MPA and PAN, demonstrate that PAN and MPA are likely separate disorders. It was not until 1994 that histologic criteria to distinguish PAN from MPA were defined at the international Chapel Hill Consensus Conference (CHCC).^[7] According to the CHCC criteria, the presence of vasculitis in arterioles, venules, and capillaries defines the diagnosis of MPA (although small- and medium-sized arteries may also be involved in MPA) and excludes the diagnosis of PAN. (See Clinical, Differentials, and Workup.)

Stages

PAN is divided into subacute, acute, and chronic stages. In the subacute stage, infiltration of mononuclear cells becomes more prominent, while in the acute stage, polymorphonuclear neutrophils infiltrate all layers of the vessel wall. (See Etiology.)
In the chronic stage, fibrinoid necrosis of the vessels causes thrombosis and tissue infarction. Aneurysmal dilatations of the involved arteries, as large as 1 cm in size, are characteristic findings of PAN. Kidney lesions show predominant arteritis without glomerulonephritis; however, in patients with severe hypertension, glomerulosclerosis may be superimposed with glomerulonephritis. Pulmonary arteries are not involved, and bronchial artery involvement is uncommon.

Patient education

Patients should understand that PAN can be a progressive systemic disease, and further complications and the involvement of other organ systems are quite common. Many patients attempt to discontinue their medications after initial symptomatic improvement, owing to the potential for adverse effects. Therefore, the benefits of medical treatments should be discussed clearly with the patient, in addition to the risks associated with the long-term use of immunosuppressants. The use of these medications necessitates close monitoring for many years to come. (See Treatment and Medications.)