Archive for March, 2010
Felty’s syndrome is defined by a clinical triad of:
seropositive rheumatoid arthritis (RA)
neutropenia (<2000/uL)
splenomegaly
. . . and, on occasion, anemia and thrombocytopenia.
Develops in individuals with long standing RA
Rare in African-Americans
Hypersplenism has been blamed for the syndrome, but not all patients have splenomegaly and splenectomy does not cure all patients.
Excessive margination of granulocytes caused by antibodies to [...]
Characterized by painful swelling and thickening of the skin in the extremities, limitations of movements due to contractures, and mild muscle weakness
Labs include elevated sedimentation rate, peripheral eosinophilia, hypergammaglobulinemia, and mildly elevated CK (not always).
Histology shows marked thickening and infiltration of the deep fascia with mononuclear cells and eosinophils.
Most patients respond to glucocorticoids.
More common in women
Associated with ingestion of L-tryptophan (essential amino-acid)
Symptoms include fever and rash, arthralgia and myalgia, cough and dyspnea, and edema.
Accompanied with eosinophilia (>1000 cells/microL).
Peripheral neuropathy, myositis (with lymphocytic and eosinophilic infiltration)
Tryptophan (contaminating L-tryptophan preparations) has been implicated as a cause.
Deficiencies of C1, C2, and C4 lead to abnormalities in immune complexes and may cause a wide variety of rheumatic diseases.
C1- (C1q component is usually deficient) SLE is common and membranoproliferative glomerulonephritis may occur.
C2 – Common deficiency (1:10,000) SLE is common; (50%) discoid lupus, vasculitides, Henoch-Schonlein purpura also occur.
C4 – Total deficiency is rare (and [...]
Granulomatous vasculitis of multiple organ systems, particularly the lungs.
Similar to polyarteritis nodosa
Granulomas may be found in the tissue far from blood vessels (these are associated with eosinophilic infiltration).
Besides lungs, the skin, cardiovascular system, kidney, peripheral nerves, and GI tract may also be affected.
Severe asthmatic attacks with pulmonary infiltrates dominate the clinical picture.
Striking eosinophilia is seen [...]
This is a rare syndrome of unknown etioligy in which small blood vessels, particularly small venules, become acutely inflamed. This results in ulceration of the affected organs.
Diagnostic criteria require recurrent oral ulcerations and 2 of the following: – Recurrent genital ulcerations (painful aphtous ulcers that last 1-2 weeks), – Eye lesions (iritis, uveitis, retinal vessel [...]
A “reactive” arthritis with a high male to female ratio (20:1). The primary infectious site is usually urogenital or occasionally intestinal. Classic infection is Chlamydia urethritis.
90% of patients possess the HLA-B27 antigen.
Diagnosis of Reiter’s syndrome should be used only for patients who have classic triad (uveitis, urethritis, and arthritis).
The arthritis usually involves the large joints [...]
Systemic vasculitis with palpable purpura
Most patents are 4-7 years of age.
Presumptive mechanism – immune complex deposition
IgA is most commonly seen in the complexes.
Symptoms related to skin, gut and joints are present in 50% of pediatric patients.
In adults 70% have skin symptoms but only 20% have gut and joint symptoms.
Renal failure is rare but still the [...]
It is granulomatous vasculitis of upper and lower respiratory tract and glomerulonephritis (variable degrees of diffuse vasculitis also may be present).
Upper respiratory symptoms (sinusitis), lower respiratory symptoms (multiple bilateral lung lesions – necrotizing granulomas), and glomerulonephritis are main symptoms.
Cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA)can be detected in majority of patients.
Demonstration of necrotizing granulomas in appropriate tissue [...]
This is a syndrome of immotile cilia associated with situs viscerum inversus.
There is also: – male infertility due to immotility of the sperm, – recurrent or chronic sinusitis, – bronchiectasis.
Granulomatous vasculitis of multiple organ systems, particularly the lungs
Similar to polyarteritis nodosa
Granulomas may be found in the tissue far from blood vessels (these are associated with eosinophilic infiltration).
Besides lungs, the skin, cardiovascular system, kidney, peripheral nerves, and GI tract may be affected.
Severe asthmatic attacks with pulmonary infiltrates dominate the clinical picture.
Striking eosinophilia is seen in [...]
Flu-like symptoms, non-productive cough, fever, malaise, dyspnea of 2 – 4 months duration. Resistant to antibiotics treatment. Inspiratory crackles and occasional wheezing can be found.
Elevated WBC or ESR or both
It is caused by excessive proliferation of granulation tissue within small airways and alveolar ducts, and chronic inflammation in the alveoli. Lung biopsy shows fibrosis with [...]
It occurs in 1 in 600 patients and is more common in whites (rare in Blacks, Japanese).
There are frequent upper respiratory tract symptoms, and chronic diarrhea can occur.
Increased incidence of asthma. Increased incidence of rheumatoid arthritis and SLE.
Antibodies to IgA–WATCH for TRANSFUSION reactions! Autosomal Dominant
This encompasses a wide range of entities (chondromalatia patellae, patellar subluxation, patellofemoral arthritis, etc.) Common causes are trauma, overuse (jumpers knee), poor patellar alignment, and poor congruency between patellar and femoral articulation surfaces.
Patients complain of knee pain in the anterior aspect, scratching “noise” or sensation and swelling.
Examination shows abnormal alignment (Q angle), patellar crepitation with [...]
This is compression neuropathy of the lateral femoral cutaneous nerve as the nerve exits the pelvis in the groin and enters the thigh. Obesity, scar in the area, and tight clothes are common causes.
Patients have neuritic, burning pain or numbness in middle third of the lateral aspect of the thigh.
Examination of hip, back and scroiliac [...]
This represents degenerative changes of the articular cartilage in the hip. Obesity, family history, injuries and long standing gait disturbances are predisposing factors.
Patients complain of groin or thigh pain and diminished mobility.
Physical examination shows limitation of external rotation, inability to cross legs (or remove socks), and tenderness 2-4 cm below the inguinal ligament.
If hip is [...]
This bursa is located between superior trochanter and median gluteal tendon. Repetitive flexion of hip and direct pressure are aggravating factors for this bursitis.
Symptoms are identical to those of trochanteric bursitis: Pain over hip and outer aspect of thigh and difficulties walking.
Examination reveals tenderness above greater trochanter, aggravation with hip abduction against resistance and in [...]
It is inflammation of the bursa between greater trochanter and iliotibial tract. Overuse of gluteal musculature and pressure over trochanteric region are most common causes.
Patients complain of hip pain and pain over outer thigh as well as difficulties walking.
Examination reveals localized midtrochanteric tenderness, aggravation of pain by abduction of hip against resistance and at extreme [...]
It is pain due to abnormal function of the lumbosacral (LS) nerve root or nerves of LS plexus (due to variety of causes of compression of the nerve) and it is characterized by progressive sensory, sensorimotor, and sensorimotor visceral abnormalities).
Patients complain of buttock pain traveling variable distance down the posterior and lateral aspects of the [...]
It is a spasm and irritation of the muscles that support lumbar spine, and is due to overuse, osteoarthritis, radiculopathy, compression fracture, scoliosis, spondylolisthesis, etc.
Patients complain of pain and stiffness that is well localized to the lumbar area.
Examination reveals muscle tenderness and spasm, straightened lumbosacral lordosis, limitation of lumbosacral flexion, and lateral bending.
A normal neurologic [...]
It is inflammation of cartilage of the chest wall at costohondral or sternohondral junctions. Most are idiopathic (Tietze syndrome), but some occur after surgery (notably after open heart surgery).
Patients present with chest pain that is well localized to chest wall.
Examination is characterized by localized tenderness at costohondral or strenohondral junctions, aggravated by pressure and easily [...]
It is an inflammatory arthritis presenting in variety of ways but classically as a symmetric, polyarticular, small joint arthritis (affecting metacarpophalangeal (MCP), proximal interphalangeal (PIP) and metatarsophalangeal joints (MTP)).
There are also monoarticular, pauciarticular, and palindromic (changing joints) forms.
All are characterized by marked synovitis and synovial thickening.
Patients present with joint stiffness in the morning, joint swelling, [...]
It is progressive fibrosis of the palmar fascia enveloping flexor tendons (typically at fourth and fifth finger) that is idiopathic in origin.
Patients complain of finger stiffness, thickening of the palm, and loss of motion of affected fingers.
Examination is characterized by painless palmar nodules, fixed flexion contractures of affected fingers, and notable absence of inflammation.
These characteristics [...]
It is an abnormal collection of tenosynovial fluid within the tendon or just adjacent to it due to microtrauma.
Patients complain of little nodule (3-5 mm) that may be mildly tender and is usually located on the palm, or the back, of the hand.
Examination shows a smooth, firm, small nodule in the palm, minimal tenderness, and [...]
It is inflammation of the flexor tendons of the fingers as they cross metacarpophalangeal (MCP) joint due to overuse (repetitive gripping and grasping). Swelling causes finger to lose smooth movement and instead catch and lock (trigger).
Patients complain of either pain or loss of smooth movement.
Examination reveals local tenderness at the MCP joints, aggravated with extension [...]
It is compression neuropathy of the median nerve, most commonly at the transverse carpal ligament but sometimes also at proximal forearm (at the pronator teres muscle).
Patients complain of intermittent loss of sensation in first three digits (most commonly), but also of forearm and wrist pain and weakness of grip (most advanced cases).
Examination is characterized by [...]
It is a very common manifestation of osteoarthritis and leads, over a long time period, to bony enlargement, loss of range of motion, and progressive subluxations.
Patients complain of pain swelling or bony enlargement particularly at the base of thumb.
Physical examination may reveal tenderness on pressure over the joint, crepitations with movement, pain aggravation in extreme [...]
It is an inflammation of the extensor and abductor tendons of the thumb (snuffbox tendons) due to overuse by friction over the radial styloid and may progress to stenosing (fibrosing) tenosynovitis.
Patients complain of sharp pain in the wrist and difficulties with gripping.
Examination shows localized tenderness over the radial styloid, and pain aggravated by thumb abduction [...]
It is an abnormal accumulation of synovial fluid in subcutaneous tissue with subsequent cyst wall formation around the fluid.
Patients notice painless lump at the wrist.
Examination reveals a highly mobile, fluctuant cyst, with minimal tenderness and normal wrist motion in most cases.
Clear non-bloody aspirate from the cyst is diagnostic.
If patient is not bothered, observation may suffice [...]
It is inflammation of the bursa olecranii (located between olecranon and skin) commonly due to repetitive trauma and about 5% each by gout and Staphylococcus aureus infection.
Patients complain of pain and marked swelling over the olecranon (swelling is usually very prominent but sometimes may be minimal).
Examination is characterized by swelling, redness, increased surface temperature, and [...]
It is injury and inflammation of common extensor tendon at lateral epicondyle of the humerus (usually due to overuse).
Patients complain of pain and weakness of the forearm (pain may be localized or more diffuse).
Examination shows local epicondylar tenderness, aggravation of pain with wrist extension, and radial deviation of hand against resistance. However, the full range [...]
It is inflammation of the long head tendon in the bicipital groove, which can lead to rupture (10-12% of cases – the highest of any tendon in the body).
Patients complain of pain in anterior aspect of the shoulder that is aggravated by lifting or overhead pushing and pulling.
Examination reveals localized tenderness over the bicipital groove, [...]
It is inflammation, osteoarthritis of AC joint, or strain, partial or complete rupture of coracoacromial and/or coracoclavicular ligament (leading to 1-degree, 2-degree and 3-degree separation respectively).
Patients complain of pain and swelling at the AC joint.
Examination will reveal point tenderness at the joint, swelling, and sometimes AC joint widening with traction. Pain may be aggravated by [...]