chap 6 symp Flashcards
Raynaud phenomenon
Scleroderma: Episodic vasoconstriction of the a’s of the extremities.
Synovitis
Sjogren syndrome
Obliterative phlebitis
IgG4 Related Disease: inflamm results in pemanent closure of vessel
increased incidence of tumors
Ataxia-telangiectasia synd
women aged 50-60
Sjogren syndrome, Scleroderma
Coronary a. dz
SLE: can lead to angina or MI.
adverse side effects of HAART
- Lipoatrophy (loss of facial fat)
- Lipoaccumulation (excess fat deposition centrally)
- Elevated lipids
- Insulin resistance
- Peripheral neuropathy
- Premature cardiovascular kidney and liver disease
Dryness of the nasal mucosa and epistaxis.
Sjogren syndrome
Peripheral neuropathy
Sjogren syndrome
edema, erythema, scaliness, follicular plugging, and skin atrophy surrounded by an elevated erythematous border; scarring
SLE: Chronic discoid lupus erythematosus
Wide spread damage to small blood vessels
Scleroderma
increased PTT
SLE
Dysphagia
Scleroderma: from esophageal fibrosis that leads to hypomotility
prone to developing AI dis’s
Brutons Agammaglobinemia, Isolated IgA deficeincy
Progressive interstitial and perivascular fibrosis in the skin(early) and multiple organs like the GI tract, kidneys, heart, muscles, and lungs later
Scleroderma
Renal:
• Mild proteinuria
• Malignant hypertension
Scleroderma
middle-aged and older men
IgG4 Related Disease
Neuropsychiatric symptoms
SLE Due to non-inflammatory occlusion of small vessels
increased serum IgG4
IgG4 Related Disease
Diffuse pulmonary fibrosis
Sjogren syndrome
Keratoconjunctivitis
Sjogren syndrome: blurring of vision, burning, and itching.
defect in ability of helper T cells to deliver activating signals to B cells and macrophages.
Hyper IgM synd
susceptable to recurrent pyogenic infections. and to Pneumocystis jiroveci pneumonia.
Hyper IgM synd
recurrent infection that leads to early death.
Wiskott-Aldrich synd
Respiratory difficulty
Scleroderma
make IgM antibodies can’t produce IgG, IgA, and IgE antibodies.
Hyper IgM synd
Inflammation of the serosal membranes
SLE
- acute: fibrinous exudate
- later: thickened
eczema
Wiskott-Aldrich synd
loss of T cell imunity
DiGeorge Synd
skin rash widespread, superficial, and non-scarring
SLE: Subacute cutaneous lupus erythematosus
Splenomegaly, capsular thickening, and follicular hyperplasia.
SLE
congenital defects of <3 and great valves
DiGeorge Synd
Valvular (libman-sacks) endocarditis
SLE
- Uncommon now due to the use of steroids.
- Nonbacterial verrucous endocarditis.
immunodeficiency
Ataxia-telangiectasia synd
telangiectasia , , and.
Ataxia-telangiectasia synd (vascular malformations)
- Calcinosis
- Raynaud phenomenon
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia
CREST Synd
butterfly malar rash
SLE: on cheeks and forehead; may have on ext’s and trunk; also on uninvolved skin
false + for syphilis
SLE: d/t antiphospholipids abs
Recurrent bronchitis and pneumonitis
Sjogren syndrome
hypocalcemia and tetany
DiGeorge Synd
Increased number of infections
SLE: d/t immune system dysfunction or tx with immunosuppressive drugs.
Parotid gland enlargement
Sjogren syndrome
normal levels B cells, but low Ig’s
CVID
esp involves skin, joints, kidney, and serosal membranes.
SLE
have inherited dificicnces of early compliment components
SLE –> impaired removal fo immune complexes
low levels of both serum and secretory IgA.
Isolated IgA deficiency
Chronic interstitial fibrosis and secondary pulmonary HTN.
SLE
mixture of the features of SLE, systemic sclerosis, and polymyositis.
Mixed Connective Tissue Disease
B cells are absent or markedly decreased in the circulation
Brutons Agammaglobinemia
susceptable to recurent infections:
• Bacterial infections of the respiratory tract: Haemophilus influenzae, Streptococcus pneumoniae, or Staphylococcus aureus.
• Viruses in the bloodstream or mucosal secretions: Enteroviruses, such as echovirus, poliovirus, and coxsackievirus.
• Giardia lamblia (intestinal protozoan).
Brutons Agammaglobinemia
skin thickening
Scleroderma
Thrombocytopenia
Wiskott-Aldrich synd
assoc w autoimmune hemolytic anemia, thrombocytopenia, and neutropenia.
Hyper IgM synd
storiform fibrosis
IgG4 Related Disease (whirled like storage pattern)
Affects Hispanic and African American women more than white men
SLE
Infants: Extensive diaper rash.
SCID
Infants: Failure to thrive.
SCID
Renal: Hematuria, red cell casts, proteinuria, and sometimes present as classic nephrotic syndrome.
SLE
Xerostomia
Sjogren syndrome: difficultly in swallowing solid foods, decrease in taste, cracks and fissures in the mouth, dryness of the buccal mucosa.
Myocarditis
SLE:(inflammation of the myocardium) can lead to tachycardia and EKG abnormalities.
Abnormal gait
Ataxia-telangiectasia synd
T cell–mediated reactions are normal
Brutons Agammaglobinemia
has anas and antiphospholipids abs
SLE
Myocardial fibrosis
Scleroderma: leading to arrhythmias or cardiac failure
susceptible to recurrent, severe infections:
•Candida albicans, Pneumocystis jiroveci, Pseudomonas, CMV, varicella, and many bac
SCID
Pleuritic and pleural effusions
SLE
recurrent sinopulmonary infections and diarrhea
Isolated IgA deficiency
neurologic deficits
Ataxia-telangiectasia synd
Infants have oral candidiasis
SCID: (prominant thrush)
abn mouth, ears, face
DiGeorge Synd
acute necrotizing vasculitis
SLE
Plasma cells are absent throughout the body
Brutons Agammaglobinemia, CVID
Renal: renal tubular acidosis, uricosuria, phosphaturia
Sjogren syndrome
Non-erosive synovitis w lttle deformity
SLE
immune destruction of the lacrimal and salivary glands by lymphocytes.
Sjogren syndrome
Valve prbs
SLE(mostly to mitral and aortic) can lead to stenosis and/or regurgitation.
Serum levels of all classes of immunoglobulins are depressed.
Brutons Agammaglobinemia, CVID