Rheumatology

·         MCTD was first described as a separate entity in 1972 and is a more specific designation than UCTD. It is not the mixture or overlap of any rheumatic diseases; rather, MCTD is used specifically when features of SLE and systemic sclerosis are present with high titers of antibody to U1RNP.

Those with no definable serology and a nondescript clinical picture are defined as having UCTD. Other patients have inflammatory myositis, Raynaud phenomenon, and sclerodactyly together with very high titer antibodies to the ribonucleoprotein antigen (U1 RNP) and no anti-DNA or anti-Sm antibody. This set of findings is defined as MCTD.

·         Osteocartilaginous bodies within a joint are often termed joint mice or loose bodies and occur commonly in osteoarthritis.

Rice Bodies: Aggregates of fibrin frequently found in the synovial fluid of patients with RA.

·         A bunion (hallux valgus) is a deviation of the proximal phalanx of the great toe toward the fibular side of the foot. It can be caused by biomechanical factors (tight and pointy-toed shoes that push the proximal phalanx across the other toes), inflammatory disease (gout or rheumatoid arthritis), or abnormal alignment (usually congenital) at the first metatarsal-cuneiform joint. If the cuneiform is abnormal, the first metatarsal may deviate excessively toward the midline (a primary varus deformity), which leads to a valgus deformity (lateral deviation) of the great toe when the abnormal foot is placed into standard shoes.

·         Caplan's syndrome: rheumatoid arthritis (RA) with pneumoconiosis.

Still's disease is a subset of juvenile RA that has systemic features (fever, lymphadenopathy, pleuropericarditis, hepatosplenomegaly, and leukocytosis) as major manifestations.

Felty’s Syndrome: SAUL (Splenomegaly, Arthritis, Ulcers (leg), Leukopenia)

Cogan's syndrome: an unusual vasculopathy associated with interstitial keratitis, sensorineural hearing loss, tinnitus, and vertigo. Systemic features such as fever, weight loss, and fatigue are present in about one-half of patients.

·         SAPHO syndrome (synovitis, acne, pustulosism hyperostosis) is a representative example of a group of syndromes that produce seronegative asymmetric arthritis usually with chest wall pain (sternoclavicular hyperostosis), chronic aseptic osteomyelitis, and severe acne. Sacroiliac joint involvement can occur and is usually unilateral. There is no HLA B27 association.

·         POEMS syndrome is a plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes, which may resemble scleroderma.

·         "string" and "mucin clot" tests: The primary component of joint fluid is hyaluronic acid. It is quite viscous and makes a "string" when expressed from a syringe as a single drop. Dilute acetic acid causes hyaluronate and protein to clump and fall to the bottom of a test tube (producing the famous "mucin clot"). Inflammatory mediators cause fragmentation of the hyaluronate-protein complex, rendering it unable to form a good mucin clot.

·         Serum transaminases (ALT, AST) are found in muscle. Therefore, elevated LFTs, especially when no concomitant gamma-glutamyl transferase is available, may reflect muscle inflammation in a patient with rheumatic disease. Check the creatine phosphokinase level.

·         Gelling describes the achiness and stiffness that occurs in patients with RA after a period of inactivity (such as getting up from the dinner table or rising from a seat after a movie). The stiffness that occurs on rising from bed in the morning is also a form of gelling.

·         The synovial lining is a thin, delicate structure (only one or two cells thick) and contains two types of synoviocytes: type A (macrophage-like cells probably derived from bone marrow) and type B (fibroblast-like cells that are probably of mesenchymal origin, secrete synovial fluid). The subsynovium constitutes the second layer of normal synovium.

·         Pannus is the term used to describe the area of proliferating synovium that meets the articular cartilage. It is believed to be the source of erosive damage in RA.

·         Antibodies directed against the citrullinated portion of certain molecules (filaggrin and fibrin among others) have been found in the sera of patients with RA and are called anti-cyclic citrullinated peptide antibodies. They are present in about 66% of RA patients and in fewer than 5% of control subjects making them quite specific for RA. Their clinical usefulness seems to be in helping to distinguish true RA in patients who are otherwise seronegative, or if the exact diagnosis of an inflammatory arthropathy is otherwise unclear.

·         Signs and symptoms of RA subside in approximately 70% of women during pregnancy. No data suggest that RA has a detrimental effect on the fetus.

·         Pregnancy and SLE:

1.       Fertility is unaffected by the disease.

2.       Although recent data suggest that pregnant patients with lupus do not have disease flares more frequently than nonpregnant patients, disease exacerbations during pregnancy do occur. Because such flares can be severe, patients with SLE should be considered at high risk. Active disease during the antecedent 3-6 months may increase the risk of a flare.

3.       Preeclampsia occurs more frequently in pregnant patients with lupus. There is also increased risk of miscarriage, abortion, intrauterine growth delay, and prematurity in patients with SLE compared with controls.

4.       The Ro antibody crosses the placenta and is responsible for most of the neonatal lupus syndromes, including skin manifestations and congenital heart block.

·         Jaccoud's deformity: Deformities of the hands secondary to chronic inflammation of the joint capsule, ligaments, and tendons. The changes may mimic those of RA (ulnar deviation of the fingers, MCP joint subluxation). Erosions are not present, although after several recurrences, notches may be seen in x-rays on the ulnar side of the metacarpal heads. Early in the course, patients can correct these changes voluntarily. Although originally described in rheumatic fever, this disorder has been extended to include the arthropathy in other conditions, most commonly SLE.

·         Pyoderma gangrenosum consists of skin lesions that begin as pustules or erythematous nodules and break down to form spreading ulcers with necrotic, undermined edges. It is associated with IBD but also occurs in chronic active hepatitis, seropositive RA (without evidence of vasculopathy), leukemia, and polycythemia vera. Differential diagnosis of the lesions includes necrotizing vasculitis, bacterial infection, and spider bites.

·         The role of genetic factors in rheumatic disease is an area of vigorous research. OA clearly has a hereditary component. Perhaps the most recognized feature is the presence of Heberden's nodes in mothers and sisters. Recent studies have uncovered a mutation in a type II collagen gene (Arg519 to Cys) that predisposes to early OA.

·         Vacuum sign: A radiographic sign of intervertebral osteochondrosis. Radiolucencies represent gas (nitrogen) that appears at the site of negative pressure produced by abnormal spaces or clefts. Clefts are produced by degeneration of intervertebral disc, especially the nucleus pulposus.

·         DILS (diffuse infiltrative lymphocytosis syndrome) is a condition occurring in between 3% and 8% of HIV-infected patients. Although it produces profound salivary gland enlargement and symptoms of dryness (sicca), it is a distinct entity with different immunogenetics and pathophysiology from Sjögren's syndrome. African American DILS sufferers show a high incidence of HLA-DR8, while DR6 and DR7 are more prevalent in Caucasians. By contrast, in patients with Sjögren's syndrome, HLA DR2 and DR3 predominate.

·         When patients with established rheumatoid arthritis have fever and an apparent flare, joint infection should be excluded by joint aspiration because septic arthritis occurs more frequently in such patients.

·         IgM rheumatoid factor is most commonly detected; IgG and, less frequently, IgA rheumatoid factors are also sometimes found. The presence of IgG rheumatoid factor is associated with a higher rate of systemic complications (e.g., necrotizing vasculitis).

·         Unlike acute inflammatory arthritides (e.g., gout or septic arthritis), RA tends not to cause marked erythema, and swelling usually does not extend far beyond the articulation. Classically, RA is symmetrical.

·         RA Rx: Advancement from NSAIDs to second-line agents is recommended if (1) symptoms have not improved sufficiently after a short trial of NSAIDs, (2) the patient has aggressive seropositive disease, or (3) there is radiographic evidence of erosions or joint destruction.

The trend today is for more aggressive treatment, and the majority of patients require additional pharmacotherapy. Most patients require rapid advancement from NSAIDs to a second-line agent, most often methotrexate.

Infliximab is used in combination with methotrexate; this appears to permit long-term use of infliximab with less formation of neutralizing antibodies. Infliximab is administered by intravenous infusion; the recommended dose is 3 to 10 mg/kg every 8 weeks. As with etanercept and all TNF inhibitors, the drug must be used with care in the presence of infections. The conventional wisdom is that glucocorticoids neither alter the course of the disease nor affect the ultimate degree of damage to joints or other structures.

To prevent the development of erosive joint changes, the standard of treatment for rheumatoid arthritis has become much more aggressive aka Inverted Pyramid Approach.

A favorable course and long remissions are associated with age less than 40 years, acute onset restricted to a few large joints, disease duration less than 1 year, and negative test results for rheumatoid factors. An unfavorable prognosis is associated with insidious onset, constitutional symptoms, the rapid appearance of rheumatoid nodules, the appearance of bone erosions early in the course of disease, and high titers of rheumatoid factors.

·         Differentiation of the Scleritis & Episcleritis is based on the more violaceous hue of the sclera in scleritis—caused by inflammation around the sclera vessels—and pain on pressure over the closed lid onto the globe, which is not seen in episcleritis. Confirmation can be done by slit-lamp examination.

·         Patients with hepatitis C infection may have polyarthralgias or polyarthritis that can resemble rheumatoid arthritis. To make matters even more problematic, rheumatoid factor is present in many patients with hepatitis C, especially in the setting of mixed cryoglobulinemia. The rheumatoid factor, as part of the cryoglobulin, may not be present in the serum if it is collected and allowed to clot at room temperature. Cryoglobulins will aggregate and clot if subjected to temperatures generally lower than 100.4° F (38° C). If rheumatoid arthritis is suspected, the specimen should be allowed to clot in a 38° C water bath and then checked for rheumatoid factor. Patients with hepatitis C should in general avoid potentially hepatotoxic drugs such as methotrexate.

·         Only those patients whose clinical presentation and examination are consistent with ankylosing spondylitis but whose radiographic testing is negative should undergo HLA-B27 testing in c/o AS.

·         In ReA, Early treatment of genitourinary infections with appropriate antibiotics (tetracycline or erythromycin) has been shown to reduce the likelihood of subsequent reactive arthritis; however, even early antibiotic use in patients with gastroenteritis does not appear to prevent reactive arthritis.

·         An inflammatory arthropathy attributable to psoriasis appears in 5% to 7% of patients with the skin disease, especially in those whose nails are affected. In general, there is little relation between joint disease and the severity of skin involvement. In fact, psoriatic skin lesions may be found only after careful scrutiny of scalp, umbilicus, or gluteal regions, and nail pitting or other changes may be the only clues supporting a diagnosis of psoriatic arthritis. Asymmetrical oligoarthritis of both small and large joints is the most common form of psoriatic arthritis. Involvement of the distal interphalangeal joints and sausage-shaped toes or fingers are highly suggestive signs. A disparity is often noted between clinical appearance and subjective symptoms; overtly involved joints may be largely asymptomatic, unlike the concordance usually found in rheumatoid arthritis. A characteristic change is the whittling of the distal ends of phalanges, giving the joints a so-called pencil-in-cup appearance, which is radiographically distinctive for psoriatic arthritis. Periostitis, bony erosions, and joint effusions are also common and so are diagnostically useful.

·         The differential diagnosis of unilateral uveitis includes ankylosing spondylitis, Reiter syndrome, and inflammatory bowel disease. Many cases, however, are idiopathic. Sarcoidosis typically causes bilateral uveitis.

·         Scleroderma renal crisis is a dreaded complication of diffuse scleroderma. It can occur rapidly and is more likely to be seen in patients with rapidly progressive skin disease. Hypertension of new onset, in conjunction with proteinuria and microscopic hematuria, is highly characteristic.

·         Inflammatory myopathy: Autoantibodies that are in large part directed against cytoplasmic ribonucleoproteins have been designated as myositis-specific autoantibodies (MSA) and are present in 30% of the patients. These antibodies tend to correlate with some specific clinical presentations, responses to therapy, and prognoses. Three groups of patients can be defined by the MSA specificities. The first group is defined by the presence of antibodies directed against aminoacyl-tRNA synthetases. These patients are generally characterized by an acute onset of muscle disease, with a high incidence of associated interstitial lung disease. They may also have arthritis and a hyperkeratotic rash on the hands, known as mechanic’s hands. This description fits the patient presented in this case. The second group includes patients with anti-SRP antibodies; these patients tend to have an abrupt onset of weakness, and they may have cardiac disease. The third group is identified by the presence of antibodies against Mi-2; these patients have a dermatomyositis with the so-called shawl sign.

·         Patients with polymyositis frequently have extramuscular manifestations. One of the most common is pulmonary fibrosis. Almost 70% of patients with pulmonary fibrosis will have the autoantibody Jo-1 in their serum. Anti–Jo-1 is one of the antisynthetase antibodies currently found only in patients with myositis. Besides pulmonary fibrosis, the antisynthetase syndrome includes Raynaud phenomenon, polyarthritis, and, in some cases, so-called mechanic’ s hands.

·         Biopsy of the muscle showing type 2 fiber atrophy is typical of steroid myopathy.

·         Cancers of the ovary, lung, lymphatic system, and hematopoietic system are overrepresented in patients with dermatomyositis. In women older than 40 years with dermatomyositis, the risk of ovarian cancer is 20 times that of the general population. Ovarian tumors are notoriously hard to find early. The most recent recommendations for detecting them in patients with dermatomyositis include a careful gynecologic examination, measurement of CA-125, and transvaginal ultrasound at 3- to 6-month intervals.

·         CSS differs most strikingly from WG in that the former occurs in patients with a history of atopy, asthma, or allergic rhinitis, which is often ongoing. In the prevasculitic atopy phase, as well as during the systemic phase of the illness, eosinophilia is characteristic and often of striking degree (≥ 1,000 eosinophils/mm3). When eosinophilia is present in WG, it is usually more modest (~500 eosinophils/mm3). Chronic sinusitis can be seen in both CSS and WG, although it is more characteristic in the latter than the former. Polymyalgia rheumatica is not associated with either CSS or WG; there is, however, a clear association between polymyalgia rheumatica and temporal arteritis. A preceding streptococcal or viral infection has been seen occasionally with both WG and CSS.

·         In GOUT, At least half of initial attacks occur in the first metatarsophalangeal joints (a condition known as podagra), but other joints of the foot may be involved simultaneously or in subsequent attacks.

Agents available for terminating the acute attack include colchicine, NSAIDs, adrenocorticotropic hormone (ACTH), and corticosteroids. Corticosteroids and ACTH have been used more often in recent years in patients with multiple comorbid conditions, because of the relatively low toxicity profile of these agents.

·         Most of the association of obesity with osteoarthritis of the knee appears to be related to environmental, rather than genetic, factors. An association between increased bone density and osteoarthritis has been noted in several studies. Women with osteoporosis and hip fractures have a decreased risk of osteoarthritis, and those affected by osteoarthritis have significantly increased bone density. This negative association suggests that soft subchondral bone absorbs impact and protects articular cartilage better than dense bone. Many patients with osteoarthritis have a family history of the disorder, and multiple genetic factors may be responsible in various forms of osteoarthritis. In women, osteoarthritis with finger joint involvement is probably the best-recognized form of arthritis with familial associations, but hereditary factors are also important in osteoarthritis of the hip.

Erythema and warmth are unusual and should suggest the presence of coexistent crystal-induced inflammation or other conditions.

Erosive osteoarthritis is characterized by polyarticular involvement of the small joints of the hand and tends to occur more often in middle-aged and elderly women.

·         For the treatment of acute back pain, NSAIDs and mild analgesics may be useful for symptom control. Muscle relaxants and opiates should be used sparingly. Spinal manipulation or specific exercise programs may also be effective in acute back pain. Over 90% of patients will improve within 1 month. Strict bed rest should be kept to a minimum, and continuation of normal activities should be enforced.

The management of chronic back pain is complex. Patients should undergo physical therapy, an exercise program, and an education program that emphasizes proper ergonomics for lifting and other activities. Light normal activity and a regular walking program should be encouraged. Encouraging the patient to apply for disability before trying different therapeutic interventions is not appropriate. Judicious use of NSAIDs and mild analgesics may improve patient function and outcome.

·         Plantar fasciitis is one of the most common causes of hindfoot pain. Patients report pain over the plantar aspect of the heel and midfoot that worsens with walking. Localized tenderness along the plantar fascia or at the insertion of the calcaneus is helpful in diagnosis. Plantar fasciitis is associated with obesity, pes planus, and activities that stress the plantar fascia. It may also be seen in systemic arthropathies such as ankylosing spondylitis and Reiter syndrome. Although radiographic spurs in the affected area are common, they may also be seen in asymptomatic persons and are therefore not diagnostic.

·         Fibromyalgia = tendon insertions and muscles hurt all the time

o   multiple tender trigger points (11/18 for Dx)

o   Amytriptyline is the treatment → because of ↑↑↑ incidence of depression

There are four principal categories of pain: nociceptive, neuropathic, psychogenic, and chronic pain of complex etiology. Chronic pain of complex etiology is the type of pain characteristic of fibromyalgia. Fibromyalgia patients often have fixed beliefs that minor traumatic events, pathogens, chemicals, or other physical agents caused their illness.

Evidence of synovitis (e.g., joint effusion, warmth over the joint, pain on joint motion), objective muscle weakness, or other definite physical or neurologic signs suggest the presence of either comorbid disease or an alternative diagnosis. Eighteen specific tender points have been identified in fibromyalgia. A patient with fibromyalgia will have pain, not just tenderness, on palpation at many of these tender points. Palpation is performed with the thumb, using approximately 4 kg of pressure—about the pressure necessary to blanch the examiner’s thumbnail. Attempting to confirm pain at all 18 tender points is not necessary for diagnosis and is inconsiderate toward patients, many of whom find tender-point palpation quite distressing. Useful tests in fibromyalgia include the following: ANA, CBC, ESR, CRP, TSH, CK, AST, and ALT. Tests for Lyme disease, Epstein-Barr virus infection, and endocrinologic status are usually unnecessary.

·         Polymyalgia rheumatica = when the shoulder girdle is the weakest

o   Pain in the shoulder girdle (actions of waving, combing hair)

o   Tender trigger points

o   very high incidence of Temporal Arteritis (Giant cell)

·         temporal headache

·         very high ESR

·         Rule of 60’s

·         age>60 years old

·         ESR >60 sed rate

·         Need more than 60mg of Prednisone to treat it.

·         Diagnose with temporal artery biopsy (MUST DO)

·         Complications – blindness due to involvement of ophthalmic artery.

·         Calcium oxalate deposition disease is usually seen in patients with end-stage renal disease; calcium phosphate deposition disease causes calcific tendinitis or Milwaukee shoulder.