diseases Flashcards
Review useful information on rheumatic diseases in order to provide effective management.
IgG4 related disease (IgG4RD) may affect what systems?
IgG4RD may involve salivary and lacrimal glands, autoimmune pancreatitis, cholangitis, retroperitoneal fibrosis, aortitis and periaortitis, thyroid disease, lung and pleural disease, and renal disease.
What is unique about IgG4 antibodies?
The disulfide bonds linking the heavy chains in IgG4 can link multiple molecules together and form unusual immune complexes. Levels of IgG4 above135 mg percent occur in about 90% of patients with IgG4RD.
What are the consensus criteria for diagnosis of Sjogren’s syndrome?
The American European consensus group definition for Sjogren’s syndrome includes: 1. Ocular symptoms of inadequate tear production 2. Ocular signs of formula damage due to inadequate tearing. 3. Oral symptoms of decreased saliva production 4. Salivary gland histopathology demonstrating foci of lymphocytes. 5. Tests indicating impaired salivary gland function. 6. SSA/anti-Ro, SSB/anti-La autoantibodies.
What gastrointestinal symptoms can be attributed to Sjogren’s disease?
Sjogren’s syndrome often causes nausea, epigastric pain, and dyspepsia. Atrophic gastritis from CD4+ T cell infiltration can result in pernicious anemia. Celiac disease is increased similar to diabetes and autoimmune thyroid disease.
What is different about HIV associated parotitis and Sjogren’s syndrome?
The infiltrate in HIV-associated disease consists of cytotoxic CD8 T cells, and Sjogren’s infiltrate are 75% helper CD4 T cells.
The sensation of eye dryness correlates best with what corneal abnormality?
Dryness correlates best with increased friction of the upper lid moving over the cornea.
What is the cause of dysfunctional tear syndrome?
Breakup of the mucus layer with subsequent decrease in goblet cell function, dryness and activation of metalloproteinases.
What skin changes can occur in Sjogren’s syndrome?
Skin involvement in Sjogren’s syndrome can include xerosis, cutaneous vasculitis, Raynaud’s phenomenon, and annular erythema,
What changes occur early by MRI in the wrists of patients with scleroderma?
Lunate edema with effusions in the intercarpal joints and radio-carpal and ulnar-carpal joints. doi: 10.1186/ar4128
How do you order lymphocyte subsets at the VA
Go to blood tests and then order flow cytometry. you need to order a CBC at the same time. need to request leukemia/lymphoma panel. At upstate you order lymphocyte subsets, no further details required.
What do elevated platelet factor 4 plasma levels mean in systemic sclerosis?
Elevated plasma levels of platelet factor 4 (CXCL-4) correlate with pulmonary fibrosis and pulmonary hypertension in systemic sclerosis.
What are the typical features of adult Still’s disease?
Still’s disease usually involves the knees, ankles, wrists more than the small joints and is often associated with pharyngitis, and lymphadenopathy. Biopsy shows B cell hyperplasia. Hemophagocytic syndrome is a rare complication and accounts for most deaths. Interstitial lung disease and myocarditis can happen. White cell counts over 15,000 and elevated ferritin levels are common. Biopsy of the skin rash shows complement deposition.
What are the diagnostic criteria for adult Still’s disease.
The Yamaguchi criteria for adult Still’s disease include four major criteria: 1. fever at least 39°C 2. arthralgia or arthritis lasting two weeks or longer 3. nonpruritic skin rash which is maculopapular and salmon colored found on the trunk and extremities during febrile episodes 4. leukocytosis exceeding 10,000 with at least 80% granulocytes. Minor criteria include sore throat, lymphadenopathy, hepatomegaly or splenomegaly, elevated AST or ALT, and negative testing for ANA and rheumatoid factor. Criteria are satisfied if at least 5 items are present including at least 2 major criteria
What are the common causes of angioedema?
Drug reaction to Ace inhibitors are the most common cause, but NSAID’s, calcium channel blockers, estrogens, fibrinolytic agents, any new medication or significant increase in dosage should also be suspect. Complement factor 1 inhibitor (C1-INH) deficiency either inherited (Types 1, 2 or 3) or acquired are not as common. Inherited forms are due to absent protein, defective protein, or inherited problems with clotting factors causing bradykinin release. Aquired C1-INH deficiency can occur with malignancies where autoantibodies develop to C1-INH.
What diseases cause arthritis?
Likely causes include: rheumatoid arthritiis, gout, Crohn’s disease, ulcerative colitis, Lyme disease, psoriatic arthritis, Reiters syndrome, ankylosis spondylitis, Systemic lupus erythematosus, reactive arthritis, acute serum sickness. Less common causes include sarcoidosis, spondyloarthritis, gonococcal arthritis, viral arthritis-parvovirus b19, post traumatic arthritis, Intraarticular hemorrhage, loose body, torn meniscus, post traumatic arthritis, and osteochondritis. Degenerative joint disease, and degenerative disc disease are more likely to cause pain and noninflammatory swelling.
What is the major complication when complete rest is used to treat tendinitis?
Without movement, tendons become weak and stiff such that whatever inflammatory reaction resolves due to rest, quickly returns when activity is resumed.
What are the 7 items included in the standard general anxiety scale?
The general anxiety disorder 7 item scale rates feeling anxious, inabliitly to stop worrying, worrying too much about different things, trouble relaxing, too restless to sit still, afraid something awful might happen, and being easily annoyed or irritable giving 0 to 3 points per item depending on frequency of daily occurrence. A total score >15 Indicates severe anxiety.
What are the red flags indicating the need for further evaluation in cases of backache of recent onset?
Red flags indicating possible definite cause for backache include: trauma, unexplained weight loss, age over 50 especially if osteoporotic, unexplained fever, history of urinary tract or other infections, immunosuppression, diabetes mellitus, history of cancer, intravenous drug abuse, long use of corticosteroids, age greater than 70, focal neurologic deficit, progressive or disabling symptoms, associated bowel or bladder dysfunction, duration longer than six weeks, and prior spinal surgery.
What life-threatening complications occur in Behcet’s disease?
The greatest morbidity and mortality comes from neurologic, ocular, and large vessel arterial or venous disease.
What medications effectively treat Behcet’s syndrome ?
Therapeutic agents that have proven effective in Behcet’s disease include glucocorticoids, colchicine, azathioprine, anti-TNF therapy, interferon alfa-2a and interferon alpha-2b at 3 to 19 million units three times weekly, thalidomide, and rituximab. Smaller trials of cyclophosphamide, cyclosporine, show some benefit as well.
What treatment is used to manage glomerulonephritis seen in Behcet’s syndrome?
Renal disease seems to behave like granulomatosis with polyangiitis, so that rituximab might be effective if glomerulonephritis is present. Those patients with amyloidosis do better with colchicine.
What commonly causes bilateral knee pain in an adolescent with normal x-rays?
Billateral anterior knee pain is a descriptive diagnosis for a condition formally called chondromalacia patella. The descriptive term is more honest as chondromalacia is difficult to demonstrate as a specific cause of knee pain and is often asymptomatic. Bilateral anterior knee pain seems to occur in late adolescence during vigorous physical activity and then subsides over years. It is not a precursor to osteoarthritis or rheumatoid arthritis. Mechanical irritation in the soft tissues guiding the quadriceps/patellar tendon from overuse and/or poor biomechanical stress tolerance is the probable cause.
What are the diagnostic criteria for chronic fatigue syndrome?
Chronic fatigue syndrome criteria include documented fatigue for six months or more, and at least four of the eight following symptoms: post exertion malaise, impaired memory/concentration, unrefreshing sleep, muscle pain, multi-joint pain without redness or swelling, tender cervical or axillary lymph nodes, sore throat, and headache. Fatigue must be severe enough to interfere with work or usual activities.
What are some local conditions that cause muscle enzyme elevation?
Localized conditions that often elevate muscle enzymes include eccentric exercise induced delayed onset muscle soreness, muscle infarction in diabetes, and iatrogenic muscle injury as from intramuscular injections.
Why are sensitive features of an illness which are non specific important to recognize?
Highly sensitive features or an illness when absent, weigh against the likelihood of that illness.
Why are specific features of an illness detected on physical examination important to recognize?
Objective specific features of an illness when present make diagnoses more certain and can help but not eliminate consideration of other causes.
What is connective tissue disease?
Connective tissue disease is one of many terms such as overlap syndrome that refer to patients who have a lupus like disorder yet do not fulfill diagnostic criteria for systemic lupus. Conditions with fatigue, arthralgia, Raynaud’s phenomena, dysphagia, and interstitial pneumonitis with a positive ANA generally fit in this category. The prognosis is good so that symptomatic treatment alone is often appropriate.
With systemic diseases can cause muscle enzyme elevation?
Systemic disorders such as inflammatory myopathy (polymyositis/dermatomyositis, systemic lupus erythematosus, and mixed connective tissue disease with antibodies to RNP), infectious myopathy, drug induced myopathies (alcohol, lipid lowering drugs, cocaine, antimalarial drugs, antipsychotic drugs, colchicine, antiretroviral drugs, ipecac, Interferon alpha and penicillamine), dystrophic myopathies, neuroleptic malignant syndrome, metabolic myopathies, hyperthermia, motor neuron disease, endocrine myopathy, and periodic paralysis should be considered. Presymptomatic myopathies as in the early stages of polymyositis, distal myopathy, mitochondrial myopathies, inclusion body myopathy, muscular dystrophy, myotonic dystrophy, and predisposition to malignant hyperthermia.
