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Pathology - Polymyositis and Dermatomyositis
illnesses of the system with no recognized etiology, though immune pathways may be involved.
Occur most frequently in women between the ages of 40 and 60.
Polymyositis (P): Associated with necrotic muscle cells, endomysium-containing lymphocytic cells, minimal vascular injury, and CDS+ T-cell damage to myofibers.
The symptoms of dermatomyositis (D) include vascular endothelial fibrosis, atrophic and necrotic muscle fibers, and immune complex deposition in blood vessels with complement activation causing an inflammatory infiltration in perivascular areas and in perimysial connective tissue of muscle fibers.
Clinical Signs and Symptoms
Polymyositis: Interstitial lung disease, arthralgias, late muscular atrophy and contracture, symmetric, gradual proximal muscle weakening.
Skin-muscle inflammation: Pediorbital edema; purplish suffusion over eyelids (heliotrope rash); scaly patches over interphalangeal and MCP joints (Gottron papules); proximal muscle weakness; interstitial lung disease; cardiac arrhythmias or dilated cardiomyopathy; dysphagia; increased risk of underlying malignancy. Characteristic dusky red rash in malar distribution mimjcking SLE.
Research results for every myositide: increased levels of aldolase and creatine kinase, as well as positive ANA, anti-Jo-1, and anti-Mi-2 antibodies.
Handling
IVIG for severe, refractory cases; high-dose corticosteroids; immunosuppression with methotrexate or azathioprine.
illnesses of the system with no recognized etiology, though immune pathways may be involved.
Occur most frequently in women between the ages of 40 and 60.
Polymyositis (P): Associated with necrotic muscle cells, endomysium-containing lymphocytic cells, minimal vascular injury, and CDS+ T-cell damage to myofibers.
The symptoms of dermatomyositis (D) include vascular endothelial fibrosis, atrophic and necrotic muscle fibers, and immune complex deposition in blood vessels with complement activation causing an inflammatory infiltration in perivascular areas and in perimysial connective tissue of muscle fibers.
Clinical Signs and Symptoms
Polymyositis: Interstitial lung disease, arthralgias, late muscular atrophy and contracture, symmetric, gradual proximal muscle weakening.
Skin-muscle inflammation: Pediorbital edema; purplish suffusion over eyelids (heliotrope rash); scaly patches over interphalangeal and MCP joints (Gottron papules); proximal muscle weakness; interstitial lung disease; cardiac arrhythmias or dilated cardiomyopathy; dysphagia; increased risk of underlying malignancy. Characteristic dusky red rash in malar distribution mimjcking SLE.
Research results for every myositide: increased levels of aldolase and creatine kinase, as well as positive ANA, anti-Jo-1, and anti-Mi-2 antibodies.
Handling
IVIG for severe, refractory cases; high-dose corticosteroids; immunosuppression with methotrexate or azathioprine.
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Pathology - Osteoporosis
caused by an increase in bone reabsorption or a deficiency in bone synthesis, which lowers bone mass.
associated with physical inactivity, genetic bone disorders, anorexia, liver disease, and endocrine abnormalities (estrogen deficit in menopause, Cushing syndrome, hyperthyroidism, and calcium deficiency).
Most frequently observed in older, Caucasian women.
Bone: Wider haversian systems, thin cortical, and thinner and fewer trabeculae characterize this qualitatively normal bone.
Clinical Signs and Symptoms
Back pain; fractures, particularly those involving the hip, femur, or thoracolumbar spine; and kyphosis, or loss of height due to spinal compression fractures.
Visualization: Reduced bone mass is shown by bone density investigations (DEXA); diffuse radiolucency is seen on radiographs.
Handling
Exercise, calcium and vitamin D supplements, selective estrogen receptor modulators (such as raloxifene), bisphosphonates (which decrease osteoclast bone resorption), and other factors.
caused by an increase in bone reabsorption or a deficiency in bone synthesis, which lowers bone mass.
associated with physical inactivity, genetic bone disorders, anorexia, liver disease, and endocrine abnormalities (estrogen deficit in menopause, Cushing syndrome, hyperthyroidism, and calcium deficiency).
Most frequently observed in older, Caucasian women.
Bone: Wider haversian systems, thin cortical, and thinner and fewer trabeculae characterize this qualitatively normal bone.
Clinical Signs and Symptoms
Back pain; fractures, particularly those involving the hip, femur, or thoracolumbar spine; and kyphosis, or loss of height due to spinal compression fractures.
Visualization: Reduced bone mass is shown by bone density investigations (DEXA); diffuse radiolucency is seen on radiographs.
Handling
Exercise, calcium and vitamin D supplements, selective estrogen receptor modulators (such as raloxifene), bisphosphonates (which decrease osteoclast bone resorption), and other factors.
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Pathology - Paget Disease of the Bone
induced by an increase in both osteoblastic and osteoclastic activity, which has been postulated to be induced by a viral infection (possibly paramyxovirus).
Gross: Can occur in one bone (monostotic) or numerous (polyostotic) and tends to involve the skull, pelvis, femur, tibia, and spine.
Microscopic: Several morphologic stages in bone: (1) Osteolytic: large osteoclasts with multiple resorption pits; (2) Osteoclastic osteoblastic: mosaic pattern of lamellar bone demonstrating both bone destruction and bone formation, both osteoclasts and osteoblasts seen; (3) Late osteosclerotic: sclerotic bone with predominance of thick trabeculae, dark mosaic lines evident.
May be asymptomatic or may manifest with bone discomfort, fractures or aberrant bone growth and deformities (increase in hat size or bowing of legs).
Complications include high-output heart failure (increased vascularity leads in numerous arteriovenous shunts), osteosarcoma, hearing loss (due to narrowing of auditory foramen), and long-bone fractures.
Imaging: Mixed thickness and lucency of bone.
Lab findings: Increased serum ALP, normal serum calcium and phosphorus, normal PTH values.
Treatment Bisphosphonates (inhibit osteoclastic activity) for symptomatic illness.
induced by an increase in both osteoblastic and osteoclastic activity, which has been postulated to be induced by a viral infection (possibly paramyxovirus).
Gross: Can occur in one bone (monostotic) or numerous (polyostotic) and tends to involve the skull, pelvis, femur, tibia, and spine.
Microscopic: Several morphologic stages in bone: (1) Osteolytic: large osteoclasts with multiple resorption pits; (2) Osteoclastic osteoblastic: mosaic pattern of lamellar bone demonstrating both bone destruction and bone formation, both osteoclasts and osteoblasts seen; (3) Late osteosclerotic: sclerotic bone with predominance of thick trabeculae, dark mosaic lines evident.
May be asymptomatic or may manifest with bone discomfort, fractures or aberrant bone growth and deformities (increase in hat size or bowing of legs).
Complications include high-output heart failure (increased vascularity leads in numerous arteriovenous shunts), osteosarcoma, hearing loss (due to narrowing of auditory foramen), and long-bone fractures.
Imaging: Mixed thickness and lucency of bone.
Lab findings: Increased serum ALP, normal serum calcium and phosphorus, normal PTH values.
Treatment Bisphosphonates (inhibit osteoclastic activity) for symptomatic illness.
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Pathology - Rickets and Osteomalacia
caused by a lack of vitamin D, which can be brought on by starvation, insufficient exposure to sunshine, fat malabsorption syndromes (induced by pancreatic insufficiency, liver illness, inflammatory bowel disease), or kidney disease (caused by a malfunction in the kidneys' ability to synthesise l,25(0H2)D).
Aluminum toxicity, phosphate shortage, and calcium deficit in the diet are additional factors.
The condition in children is called rickets, while the sickness in adults is called osteomalacia.
Pathophysiology: Lack of vitamin D reduced the kidney's and intestines' ability to absorb Ca2+, which in turn reduced the calcification of the osteoid matrix.
Increased unmineralized bone matrix in the bone; trabeculae contain a calcified bone core encircled by an unmineralized osteoid sheath.
Clinical Signs and Symptoms
Rickets symptoms include reduced stature, rachitic rosary (thickening of the costochondral junction), pigeon breast (protrusion of the sternum), late fontanelle closure, and craniotabes (thinned occipital and parietal bones).
Osteomalacia: Weakness in the muscles and diffuse bone discomfort. Imaging: A radiograph displaying diffuse radiolucency and thinning cortical bone.
Results from the laboratory for osteomalacia and rickets: reduced phosphate and calcium concentrations.
Treatments
supplementation of vitamin D; underlying cause therapy.
Renal osteodystrophy is the term used to describe osteomalacia caused by chronic renal illness that leads in a relative vitamin D shortage and secondary hyperparathyroidism.
caused by a lack of vitamin D, which can be brought on by starvation, insufficient exposure to sunshine, fat malabsorption syndromes (induced by pancreatic insufficiency, liver illness, inflammatory bowel disease), or kidney disease (caused by a malfunction in the kidneys' ability to synthesise l,25(0H2)D).
Aluminum toxicity, phosphate shortage, and calcium deficit in the diet are additional factors.
The condition in children is called rickets, while the sickness in adults is called osteomalacia.
Pathophysiology: Lack of vitamin D reduced the kidney's and intestines' ability to absorb Ca2+, which in turn reduced the calcification of the osteoid matrix.
Increased unmineralized bone matrix in the bone; trabeculae contain a calcified bone core encircled by an unmineralized osteoid sheath.
Clinical Signs and Symptoms
Rickets symptoms include reduced stature, rachitic rosary (thickening of the costochondral junction), pigeon breast (protrusion of the sternum), late fontanelle closure, and craniotabes (thinned occipital and parietal bones).
Osteomalacia: Weakness in the muscles and diffuse bone discomfort. Imaging: A radiograph displaying diffuse radiolucency and thinning cortical bone.
Results from the laboratory for osteomalacia and rickets: reduced phosphate and calcium concentrations.
Treatments
supplementation of vitamin D; underlying cause therapy.
Renal osteodystrophy is the term used to describe osteomalacia caused by chronic renal illness that leads in a relative vitamin D shortage and secondary hyperparathyroidism.
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Pathology - Scurvy
caused by a lack of vitamin C, typically from insufficient consumption.
Pathophysiology: Insufficient vitamin C leads to decreased collagen synthesis and a reduction in osteoblasts' ability to create osteoid matrix.
Bone: Reduced osteoid matrix; expansion of cartilaginous material without osteoid replacement of the epiphyseal cartilage.
Clinical Signs and Symptoms
bleeding into joint spaces, purpura and petechiae, subperiosteal hemorrhage, gingival edema, anemia, exhaustion, and poor wound healing.
Results from the lab: reduced Hct and decreased plasma ascorbic acid.
Supplemental vitamin C for treatment.
caused by a lack of vitamin C, typically from insufficient consumption.
Pathophysiology: Insufficient vitamin C leads to decreased collagen synthesis and a reduction in osteoblasts' ability to create osteoid matrix.
Bone: Reduced osteoid matrix; expansion of cartilaginous material without osteoid replacement of the epiphyseal cartilage.
Clinical Signs and Symptoms
bleeding into joint spaces, purpura and petechiae, subperiosteal hemorrhage, gingival edema, anemia, exhaustion, and poor wound healing.
Results from the lab: reduced Hct and decreased plasma ascorbic acid.
Supplemental vitamin C for treatment.
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Pathology - Osteochondroma
arises from the growth plate's lateral portion being displaced, which causes the bone to proliferate away from the long axis.
The most frequent benign tumor of the bone is osteochondroma.
Most frequently observed in guys under 25.
Gross: Usually appears in the tibia or femur metaphysis; a cartilage cap protrudes from the bone to cover the growing stalk of the bone.
Microscopic: Hyaline cartilage covers the freshly produced bone that results from endochondral ossification, which covers the medullary cavity that forms the stalk's core.
frequently asymptomatic and found by accident on radiographs; if symptomatic, it may show up as a mass or pain from a stalk fracture, nerve impingement, or irritation of the soft tissue around the mass; in rare cases, it may progress to chondrosarcoma.
Imaging: Radiographs show the growth of the bone stalk.
Surgery in case of symptoms.
several hereditary exostosis is an autosomal dominant disorder in which there are several osteochondromas.
Benign tumors originating from osteoblasts include osteoid osteoma and osteoblastoma. Osteoblastomas typically originate in the spine, whereas osteoid osteomas typically start in the femur or tibia.
The most prevalent symptom of these lesions in men under 25 is bone discomfort.
arises from the growth plate's lateral portion being displaced, which causes the bone to proliferate away from the long axis.
The most frequent benign tumor of the bone is osteochondroma.
Most frequently observed in guys under 25.
Gross: Usually appears in the tibia or femur metaphysis; a cartilage cap protrudes from the bone to cover the growing stalk of the bone.
Microscopic: Hyaline cartilage covers the freshly produced bone that results from endochondral ossification, which covers the medullary cavity that forms the stalk's core.
frequently asymptomatic and found by accident on radiographs; if symptomatic, it may show up as a mass or pain from a stalk fracture, nerve impingement, or irritation of the soft tissue around the mass; in rare cases, it may progress to chondrosarcoma.
Imaging: Radiographs show the growth of the bone stalk.
Surgery in case of symptoms.
several hereditary exostosis is an autosomal dominant disorder in which there are several osteochondromas.
Benign tumors originating from osteoblasts include osteoid osteoma and osteoblastoma. Osteoblastomas typically originate in the spine, whereas osteoid osteomas typically start in the femur or tibia.
The most prevalent symptom of these lesions in men under 25 is bone discomfort.
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Pathology - Giant Cell Tumor
harmless tumor originating from monocytes.
Typically impacts women in the age range of 20 to 40.
Gross: Typically develops from lengthy bone epiphyses, particularly at the knee (proximal tibia, distal femur); big, reddish-brown in color; frequently exhibits bleeding, necrosis, and cystic degeneration.
Microscopic: Spindle-shaped large cells of the osteoclast type, with up to 100 nuclei; fibrous stroma; uniform oval mononuclear cells exhibiting multiple mitoses.
Clinical Signs and Symptoms
fractures; joint pain and other signs of arthritis.
Rarely, metastases to the lung or lymph nodes are among the complications.
Visualization: appearance of a soap bubble or two bubbles on a radiograph.
Treatment includes radiation and chemotherapy if there are lung metastases in addition to surgical excision.
Though it recurs 40%–60% of the time after therapy, it is still aggressive and very rarely develops malignant.
Most often found in the hands and feet, enchondroma is a benign cartilaginous tumor that originates from the intramedullary bone. Enchondromas can be painful and result in a fracture, but they are typically asymptomatic and discovered by accident on radiographs (notice the distinctive O-ring sign on x-rays).
harmless tumor originating from monocytes.
Typically impacts women in the age range of 20 to 40.
Gross: Typically develops from lengthy bone epiphyses, particularly at the knee (proximal tibia, distal femur); big, reddish-brown in color; frequently exhibits bleeding, necrosis, and cystic degeneration.
Microscopic: Spindle-shaped large cells of the osteoclast type, with up to 100 nuclei; fibrous stroma; uniform oval mononuclear cells exhibiting multiple mitoses.
Clinical Signs and Symptoms
fractures; joint pain and other signs of arthritis.
Rarely, metastases to the lung or lymph nodes are among the complications.
Visualization: appearance of a soap bubble or two bubbles on a radiograph.
Treatment includes radiation and chemotherapy if there are lung metastases in addition to surgical excision.
Though it recurs 40%–60% of the time after therapy, it is still aggressive and very rarely develops malignant.
Most often found in the hands and feet, enchondroma is a benign cartilaginous tumor that originates from the intramedullary bone. Enchondromas can be painful and result in a fracture, but they are typically asymptomatic and discovered by accident on radiographs (notice the distinctive O-ring sign on x-rays).
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Pathology - Osteosarcoma
tumor produced by cancerous osteoblasts, which are cells that make bone.
Bone infarcts, fibrous dysplasia, ionizing radiation, hereditary retinoblastoma, Paget disease of the bone, and p53 mutations are risk factors.
occurs most commonly in men between the ages of 10 and 20, however the elderly have a lower peak of frequency.
Pathology
Gross: Develops in long bone metaphysis, particularly at the knee (proximal tibia, distal femur); huge, bulky mass with areas of cystic degeneration and bleeding; frequently destroys surrounding cortex and extends into medullary canal.
Microscopic: development of neoplastic osteoid formation, anaplastic cells, and multinucleated large cells.
Clinical Signs and Symptoms
Pain and edema surrounding the fracture site; frequently spreads to the liver, brain, or lung and may show signs of metastasis.
Results from the lab: elevated ALP levels in the serum.
Imaging: sunburst growth pattern on radiograph; Codman triangle (raising of periosteum).
Interventions
chemotherapy; amputation of a limb; surgical resection.
The 5-year survival rate for the prognosis is about 60%.
The most frequent primary malignant tumor of bone is osteosarcoma.
A malignant cartilaginous tumor, chondrosarcoma primarily affects men between the ages of 30 and 60.
It usually affects the tibia, femur, ribs, pelvis, and spine.
tumor produced by cancerous osteoblasts, which are cells that make bone.
Bone infarcts, fibrous dysplasia, ionizing radiation, hereditary retinoblastoma, Paget disease of the bone, and p53 mutations are risk factors.
occurs most commonly in men between the ages of 10 and 20, however the elderly have a lower peak of frequency.
Pathology
Gross: Develops in long bone metaphysis, particularly at the knee (proximal tibia, distal femur); huge, bulky mass with areas of cystic degeneration and bleeding; frequently destroys surrounding cortex and extends into medullary canal.
Microscopic: development of neoplastic osteoid formation, anaplastic cells, and multinucleated large cells.
Clinical Signs and Symptoms
Pain and edema surrounding the fracture site; frequently spreads to the liver, brain, or lung and may show signs of metastasis.
Results from the lab: elevated ALP levels in the serum.
Imaging: sunburst growth pattern on radiograph; Codman triangle (raising of periosteum).
Interventions
chemotherapy; amputation of a limb; surgical resection.
The 5-year survival rate for the prognosis is about 60%.
The most frequent primary malignant tumor of bone is osteosarcoma.
A malignant cartilaginous tumor, chondrosarcoma primarily affects men between the ages of 30 and 60.
It usually affects the tibia, femur, ribs, pelvis, and spine.
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Pathology - Ewing Sarcoma
connected to the translocation t of the chromosome (11, 22), resulting in the formation of the fusion protein EWS-FL/1, a super-activated transcriptional factor.
classified as a little, spherical, blue tumor; the source of the tumor cells is uncertain.
Most common in males under the age of twenty.
Pathology
Gross: Usually develops in the scapula, pelvis, ribs, and medullary cavities of long bones.
Microscopic: Necrosis may be seen; tumor cells may be organized in a pseudorosette pattern. Sheets of tiny, spherical, blue, homogenous cells with minimal cytoplasm.
Clinical Symptoms and Signs
painful, enlarged, expanding mass; may exhibit systemic symptoms such as fever and anemia (may resemble symptoms of osteomyelitis); may occur with pathological fracture.
As a result, there may be lung, lymph node, or infrequently, central nervous system metastases.
Imaging: On a radiograph, a lymphatic tumor resembles an onion.
Interventions
radiation, chemotherapy, and surgical resection.
An very aggressive tumor with a 70% 5-year survival rate for localized disease and less than 30% for metastatic disease is prone to metastasis early.
connected to the translocation t of the chromosome (11, 22), resulting in the formation of the fusion protein EWS-FL/1, a super-activated transcriptional factor.
classified as a little, spherical, blue tumor; the source of the tumor cells is uncertain.
Most common in males under the age of twenty.
Pathology
Gross: Usually develops in the scapula, pelvis, ribs, and medullary cavities of long bones.
Microscopic: Necrosis may be seen; tumor cells may be organized in a pseudorosette pattern. Sheets of tiny, spherical, blue, homogenous cells with minimal cytoplasm.
Clinical Symptoms and Signs
painful, enlarged, expanding mass; may exhibit systemic symptoms such as fever and anemia (may resemble symptoms of osteomyelitis); may occur with pathological fracture.
As a result, there may be lung, lymph node, or infrequently, central nervous system metastases.
Imaging: On a radiograph, a lymphatic tumor resembles an onion.
Interventions
radiation, chemotherapy, and surgical resection.
An very aggressive tumor with a 70% 5-year survival rate for localized disease and less than 30% for metastatic disease is prone to metastasis early.
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Pathology - Rheumatoid Arthritis
The etiology is uncertain, although it is thought that in individuals who are genetically vulnerable (HLA-DR4), an acute inflammatory response sets off a chronic autoimmune reaction.
most prevalent in females aged 30 to 50.
Joint: Initially, there is edema and lymphocytic infiltration in the synovium, which results in the formation of villi made of synovial lining cells and neutrophils in the synovial fluid. Later, the cartilage is destroyed and replaced with fibrocellular granulation tissue, or pannus, and joint deformity is caused by scarring following inflammation.
Rheumatoid nodule: firm, nontender, subcutaneous nodule; palisade of macrophages, lymphocytes, and fibroblasts encircling core of fibrinoid necrosis.
Clinical Signs and Symptoms
A rheumatoid nodule usually over bony prominences; a swan-neck deformity (flexion of DIP with extension of PIP joint); a boutonniere deformity (extension of DIP with flexion of PIP joint); ulnar deviation of fingers; joint deformities; fatigue; fever; symmetric joint swelling with stiffness and pain that often involves PIP and MCP joints of the fingers, wrists, knees, and ankles that is most severe in the morning and improves with activity.
Pericarditis, pleuritis, vasculitis, pulmonary fibrosis, and secondary amyloidosis are among the complications.
Imaging: narrowing of the joint space; osteopenja; bone degradation at the joints.
Results from the lab included elevated ESR, anemia, positive anti-CCP antibody, and positive rheumatoid factor (IgM antibody against IgG Fe fragment).
Treatments include NSAIDs, glucocorticoids, and immunomodulators (e.g., etanercept for severe disease; methotrexate or leflunomide for mild disease).
The etiology is uncertain, although it is thought that in individuals who are genetically vulnerable (HLA-DR4), an acute inflammatory response sets off a chronic autoimmune reaction.
most prevalent in females aged 30 to 50.
Joint: Initially, there is edema and lymphocytic infiltration in the synovium, which results in the formation of villi made of synovial lining cells and neutrophils in the synovial fluid. Later, the cartilage is destroyed and replaced with fibrocellular granulation tissue, or pannus, and joint deformity is caused by scarring following inflammation.
Rheumatoid nodule: firm, nontender, subcutaneous nodule; palisade of macrophages, lymphocytes, and fibroblasts encircling core of fibrinoid necrosis.
Clinical Signs and Symptoms
A rheumatoid nodule usually over bony prominences; a swan-neck deformity (flexion of DIP with extension of PIP joint); a boutonniere deformity (extension of DIP with flexion of PIP joint); ulnar deviation of fingers; joint deformities; fatigue; fever; symmetric joint swelling with stiffness and pain that often involves PIP and MCP joints of the fingers, wrists, knees, and ankles that is most severe in the morning and improves with activity.
Pericarditis, pleuritis, vasculitis, pulmonary fibrosis, and secondary amyloidosis are among the complications.
Imaging: narrowing of the joint space; osteopenja; bone degradation at the joints.
Results from the lab included elevated ESR, anemia, positive anti-CCP antibody, and positive rheumatoid factor (IgM antibody against IgG Fe fragment).
Treatments include NSAIDs, glucocorticoids, and immunomodulators (e.g., etanercept for severe disease; methotrexate or leflunomide for mild disease).
