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Pseudorheumatoid arthritis (Type B)
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- Relapsing polychondritis
Pseudorheumatoid arthritis (Type B)10% with low titre RF / joints inflamed “out of phase” (like gout, not like RA), osteophytes, CPPD, lack of typical erosion patterns on X-ray can mimic sepsis in elderly patients (fever, WBCs, mental status, polyarthritis) Hydroxyapatite (HA) secondary to many systemic disease states (apparently, mostly with elevated Ca2+) / crystals so small, a special stain is required to detect / anti-inflammatory treatment may shorten duration of attacks, long-term changes cannot be undone? Calcium oxalate (CaOx) strong positive (+) birefringence Primary: rare genetic disorder, death < 20 yrs Secondary: renal failure or vitamin C abuse Fibromyalgia usu. middle-aged women / hypersensitivity to physical stimulation causing pain, fatigue, poor sleep(mechanism poorly understood) Diagnosis: diagnosis by exclusion of other disorders and demonstrating ≥ 11 of 18 trigger points Labs: no specific lab abnormalities Treatment: no good treatment, but TCA’s might provide some relief Relapsing polychondritisInflammation of cartilage (breakdown of chondroitin sulfate) Findings: saddle-nose deformity, scleral thinning (scleromalacia), floppy ear, aneurysms, valvular insufficiency (AR, MR, TR), tracheal narrowing (steeple sign) Liquid lipid microspherules? Other Bone Disorders Scoliosis adolescent females > males / 20% with positive family history slipped capital femoral epiphyses 20% with referred knee pain (can be misleading) / occurs in pubescent males, happens gradually, can be bilateral / Treatment: surgical with pinning Villonodular synovitis (benign neoplasms) aggregates of polyhedral cells, hemosiderin, foam cells, giant cells, zones of sclerosis Treatment: surgery if possible, usually difficult to excise
single or multiple, diffuse involvement, red-brown projections giant cell tumor of tendon sheath (localized tenosynovitis) small, discrete nodule Bone Cancer mets most common form: BLT2KP lung > breast (lytic) > prostate (blastic) > testes, kidney primary malignant: OS, malignant fibrous histiocytoma, adamantinoma, chordoma Osteochondroma most common primary bone lesion / young males / sessile or stalked / cartilage cap / usually stops growing as bones mature Chondroma single or multiple (Olier’s Disease, Maffucci’s syndrome) / short bones of hands, feet / radiolucent [XR] / lobulated, hypercellular, disorganized / focal calcification w/in lesion / self-limited disease Chondrosarcoma - good prognosis proliferation of malignant cartilage / older males / axial skeleton / surgery only useful option Osteoid osteoma very common / young males / < 2 cm growth / appendicular skeleton / produces pain at night (relieved by aspirin) / radiolucent lesion surround by reactive bone formation / surgical removal / 25% relapse due to poor nidus locating by surgeon Osteosarcoma (OS) - poor prognosis pre-op and post-op chemotherapy / arm, leg bones / produces bone, cartilage, spindle cells usually have mets / cortical destruction w/ extension in soft tissues (Codman’s triangle) Parosteal osteosarcoma (POS) - excellent prognosis young, early middle age, women / long bones / radiolucent ‘string sign’ along cortex / spindle cells produce well-formed bone Ewing’s sarcoma (variable prognosis) small cell neoplasia / unknown histiogenesis / very young, males, lower extremities / XR: moth-eaten intramedullary pattern, ‘onion skin’ periosteal reactive bone / diaphysis to metaphysis / PAS+ cytoplasm / therapy evolving Fibrous cortical defect very common / young, males, long bones / XR: metaphysis, sub-cortical, soap bubbles, sclerosis at interface spindle cells, foamy macrophages, hemosiderin, chronic infiltrate / self-limiting at skeletal maturity Fibrous dysplasia very common / single, multiple / young, localization random / XR: radiopaque, ‘shepherd’s crook’ of proximal femur / spindle, cells, woven bone, lack of osteoblastic rimming, Chinese character appearance / no treatment unless symptomatic / excellent prognosis Malignant fibrous histiocytoma (poor prognosis) similar demographics to OS / XR: metaphysis, destructive, radiolucent / anaplastic spindle cells, storiform pattern / treatment same and prognosis slightly worse than OS Giant cell tumor of bone benign but aggressive local tumor / young, wide distribution / hemorrhage / surgery when possible / extended curettage (experimental) or resection / prosthesis / 98% monostotic / radiation contraindicated (secondary sarcomas) Adamantinoma (good prognosis) primary malignant bone tumor / young males, tibia/fibula / XR: may be multifocal (observe carefully) / epithelial or endothelial proliferation / complete surgical extirpation Chordoma malignant bone tumor arising from notochord / 40s to 60s / males / physaliferous cells in acid mucoid background / surgery and post-op radiation survival: sacral 60% (fair) 5 yr, cervical (horrible) 50% 5 yr 0% 8 yr
1 in 300 black women / HLA-DR3 / HLA-DR2 Differential: psoriasis (i.e. avoid UV light therapy), lyme disease, drug reactions, tinea Diagnosis: must meet 4 of 11 criteria (malar rash, discoid rash, photosensitivity, mucosal ulcers, arthritis, serositis, renal, neurologic, hematologic, positive ANA, positive LE or anti-ds or anti-Sm) Complications: General: fatigue, weight loss, fever Skin: malar rash (fixed erythema, flat or raised over malar area, tends to spare nasolabial folds), discoid rash (erythematous raised patches with adherent keratotic scaling and follicular plugging), photosensitivity, periungual telangiectasia, alopecia Renal: many forms possible / note: 80-90% of SLE becomes dormant when ESRD occurs mesangial (earliest: may remit or transition to other forms) focal proliferative (50%) membranous (50%) diffuse proliferative (20%, worst) Cardiovascular: endocarditis (Libman-Sacks/caused by APA syndrome) pericarditis hypercoagulability Raynaud’s (20-30%) purpuric lesions (see hematologic) Hematologic: hypercoagulable state (in addition, there is arterial-specific hypercoagulability in SLE patients due to variant mannose-binding lectin genes) leukopenia (<4000/mm3), lymphopenia (<1500/mm3), thrombocytopenia (<100,000/mm3), hemolytic anemia Pulmonary: More common pleuritis (LE cells are very specific, WBC’s with pushed aside nucleus, very characteristic appearance, but make sure pathologist looks for them), pleural effusion (mildly exudative, unilateral or bilateral) Less common ILD (including pneumonitis) PE (from APA) pulmonary HTN diffuse alveolar hemorrhage (rare): 90% will have concurrent nephritis, abrupt onset, young women, association with pneumonia) malignancy: ↑ risk of lung cancer > lymphoma other: BOOP, shrinking-lung syndrome, lymphadenopathy, infections GI: painless oral or vaginal ulcers, non-specific abdominal complaints / GI vasculitis (less common, serious) Musculoskeletal: arthralgias (symmetric/peripheral, two or more joints, swelling, effusion, tenderness but NOT erosive; only small percentage actually get joint deforming arthritis as in RA) CNS: diffuse psychosis, depression or focal neurological deficits (including seizures) [Ddx] / 50% experience some degree of neuropsychiatric problems / may see cystoid bodies in fundus Other: hepatosplenomegaly (functional hyposplenism), LAD Labs: decreased C3/C4 (can be marker of active disease, either can be depressed first depending on if classical or alternate pathway is activated, can also be decreased from poor synthesis such as in liver disease) thrombocytopenia, anemia schistocytes generally not seen without active vasculitis or major HTN anticardiolipin Ab (30-50% have it, fewer actually have APA syndrome) false positive VDRL anti-nuclear antibodies (ANA) (labs) 98% sensitivity, often high titre (1:80 happens in many people is non-specific) / 10% of SLE in whites may be ANA only (no other positive Abs), this is rare in non-whites Specific Patterns Peripheral active disease, renal involvement Diffuse SLE, RA, discoid lupus, normal elderly (rare) / can mask speckled or peripheral pattern Speckled RA, SLE, MCTD, chronic discoid lupus, chronic lung disease, scleroderma, normal elderly (rare) Nucleolar pattern scleroderma (occasionally SLE, RA, Sjogren’s) anti-ds DNA specific for SLE, can fluctuate with treatment RPGN, rash, pneumonitis anti-Sm very specific anti-Ro (SSA) Sjogren’s, SLE, myositis, etc. anti-La (SSB) cannot have La without Ro anti-U1-RNP/nRNP SLE, PSS, myositis / cytoplasmic Ab, thus can be negative ANA / very strong correlation with blacks + Raynaud’s and/or myositis and primary pulmonary HTN (uncommon) anti-ribosomal P specific for SLE, can be sole antibody with ANA negative SLE ANA to histone (diffuse pattern) suggests drug-induced (90% sensitive, but not so specific in that 20-30% of idiopathic SLE will have anti-histone Abs)
SLE-like syndrome / (+) ANA to histone / (+) genetic predisposition, ANA may remain positive for years Course: usually much less severe, resolves within 6 months of stopping drug
Pathology: interface dermatitis and granular deposition of IgG along the dermal-epidermal junction Chronic Discoid Lupus Chilblain’s Lupus – rare violaceous digital plaques, nodules develop after cold exposure / anti-Ro (SSA), Raynaud’s, changes in nail-fold capillaries / lesions contain papillary and deep dermal T-cells Pregnancy and SLE antibodies DO cross placenta and affect infant up to 6 months after birth / can cause irreversible congenital heart block (anti-Ro/SSA) (often before mother is diagnosed) Treatment: education! If disease is controlled (and < 10 mg prednisone), then it is okay to proceed with pregnancy Sjögren’s syndrome (Keratoconjunctivitis Sicca) [NEJM] females (usu. peri-menopause) / HLA-DR3 / can have primary or secondary (with SLE/RA) Mechanism: lymphocytic infiltration and destruction of lacrimal and salivary glands
Peripheral neuropathies (50-60%) / usu. pure sensory, asymmetric chronic neuropathy (may precede other symptoms by many years) GERD increased risk of B-cell lymphoma (1-5%) association with decreased PFTs (lung disease) Diagnosis: eye exam (Schirmer test), lip biopsy (salivary gland) in uncertain cases, SSA (anti-Ro), SSB (anti-La) occur in only 3% of cases [note, one does not have anti-SSB without anti-SSA], often have elevated RF, ESR Treatment: symptomatic (fake tears, benzodiazepines for anxiety, etc.), pro-cholinergic agents, steroids for severe systemic complications Polymyositis/Dermatomyositis Download 3.95 Mb. Share with your friends:
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