Causes of Interstitial Lung Disease - Differential Diagnosis Algorithm
Idiopathic Interstitial Pneumonia
• (UIP) Usual Interstitial Pneumonia - fibrosis, with relatively little inflammation, most common
• (NSIP) Non-specific Interstitial Pneumonia
• (DIP) Desquamative Intersitial Pneumonia
• (LIP) Lymphoid Interstitial Pneumonia - slowly progressive dyspnea and cough, “velcro” crackles, clubbing, worse at the bases and periphery, Poor Prognosis
Granulomatous - Sarcoidosis (Debate if this should be 1° or 2°) - Multisystem characterized by granulomatous inflammation
Primary - Other (Rare)
• Lymphangioleiomyomatosis (LAM),
• Pulmonary Langerhans cell histiocytosis (PLCH)
• Eosinophilic pneumonia
Congestive Heart Failure - Impaired cardiac function resulting in fluid leaking out of pulmonary capillaries into intersitial space
Infection - “Atypical” Pneumonia
Malignancy - Metastases to lung as nodules
Hypersensitivity Pneumonia - Immunologic (hypersensitivity) reaction to inhaled antigen
Connective Tissue Disease - Rheumatoid Arthritis, Systemic Lupus and Scleroderma
Pneumonconiosis - Lung disease (nonneoplastic) resulting from inhalation of inorganic and organic dusts
Drugs/Radiaition
• Drugs: Amiodarone, Nitrofurantoin, Methotrexate, Beomycin
• Acute or Chronic Radiation Pneumonitis - 4-12 weeks after exposure, 6-12 after.
- Amy Chung, MD, MSc @AmyChung
#Interstitial #Lung #Diseases #ILD #Causes #Differential #Diagnosis #Algorithm
Interstitial Lung Diseases (ILD)
Exposure-Related ILD (a.k.a. extrinsic allergic alveolitis):
• Hypersensitivity pneumonitis, Farmer's lung, Bird fanciers' lung, (Many others...)
• Pneumoconioses (i.e. ILD caused by inhalation of inorganic substances): Coal (coal workers' lung), Asbestos (asbestosis), Silica (silicosis), Beryllium (berylliosis)
• Radiation pneumonitis
• Medication-induced ILD: Amiodarone, Nitrofurantoin, Methotrexate
ILD Secondary to Connective Tissue Disease:
• Systemic sclerosis, Rheumatoid arthritis, Mixed connective tissue disease, Polymyositis-dermatomyositis, Lupus
Idiopathic Interstitial Pneumonias:
• Idiopathic pulmonary fibrosis (IPF), Nonspecific interstitial pneumonia (NSIP), Cryptogenic organizing pneumonia (COP), Respiratory bronchiolitis-interstitial lung disease (RB-ILD), Desquamative interstitial pneumonia (DIP), Acute interstitial pneumonia (AIP)
Other ILDs:
• Sarcoidosis
• Vasculitis: Granulomatosis with polyangiitis, Eosinophilic granulomatosis with polyangiitis (a.k.a. Churg Strauss syndrome)
• Eosinophilic pneumonia: Acute, Chronic
• Pulmonary Langerhans cell histiocytosis
- Dr. Eric Strong @DrEricStrong - Strong Medicine https://www.youtube.com/c/EricsMedicalLectures/
#Interstitial #Lung #Diseases #ILD #diagnosis #differential #pulmonary #causes
Bronchoalveolar Lavage (BAL) on Bronchoscopy
BRONCHOALVEOLAR LAVAGE is a technique used in bronchoscopy for several diagnostic purposes. For example, if there is concern for an infectious process in the right middle lobe, the scope can be wedged in one of the segments in the right middle lobe and a BAL can be done. The bronchoscope is directed into a smaller airway of concern (guided by chest imaging), and it is “wedged” into the small airway. A proper wedge is noted when the airway collapses when suction is applied.
Once wedged, sterile normal saline is inserted into a subsegment of the lung, followed by suction and collection of the instillation for analysis.
This simple procedure can help a pulmonologist in several ways as noted below!
— Infection
— Diagnosing sarcoidosis
— Diffuse Alveolar Hemorrhage (DAH)
— Pulmonary Alveolar Proteinosis (PAP)
— Eosinophilic Pneumonia
— Hypersensitivity Pneumonitis (HP)
— Some (but often not helpful) Interstitial Lung Diseases (ILDs)
— and even occupational exposures!
This is a video of a bronchoscope wedged into a subsegment of the lung with instillation of saline for the BAL.
Dr. Komal Parikh @pulmcritdoc
#Bronchoalveolar #Lavage #BAL #Bronchoscopy #clinical #video #pulmonary
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Cryptogenic Organizing Pneumonia (COP)
Cryptogenic Organizing Pneumonia Overview:
• Idiopathic form of organizing pneumonia
• formerly BOOP
• Type of interstitial lung that affects the distal bronchioles, alveolar ducts and walls.
• Secondary OP can be seen in association with CTD, drugs, malignancy and other interstitial pneumonias
Cryptogenic Organizing Pneumonia Epidemiology:
• Unknown
• 6-7 cases per 100,000 hospital admissions
• 56% of OP considered cryptogenic
Cryptogenic Organizing Pneumonia - Clinical manifestations:
• Onset: 5-6th decades of life
• History: cough, dyspnea, fever, malaise, weight loss of > 10 pounds
• Hemoptysis - rare
Cryptogenic Organizing Pneumonia - Imaging:
• CXR: Peripheral distribution of opacities, Unilateral consolidation and GG pattern, irregular lines, nodular opacities. Rare: pleural effusions, pleural thickening, cavities
• HRCT: patchy consolidation: peripheries and lower lung zone, GGO, small modular opacities, bronchial wall thickening with dilatation
Cryptogenic Organizing Pneumonia - Diagnosis:
• Lung biopsy: TBNA or Surgical lung biopsy
• Histopathology: Intraluminal plugs of inflammatory debris-small airways, alveolar ducts and adiacent alveoli
• Absence of histopathology suggestive of other processes
Workup / Laboratory Studies:
- No specific lab studies
- Make sure to order:
• CBC with diff
• Chem
• Transaminases
• UA
• Pneumonia: blood culture, sputum culture, urinary studies legionella/strep, HIV
• CTD workup: ANA, RF, CCP, CPK, anti-scl70, anticentromere ab, Anti-dsDNA, ANCA
COP DDX:
• CAP, Idiopathic interstitial pneumonia’s, Hypersensitivity pneumonitis, Granulomatous organizing pneumonia, Chronic eosinophilia pneumonia, Pulmonary lymphoma, Diffuse alveolar damage
Cryptogenic Organizing Pneumonia Treatment:
• Mild: observation
• Moderate: oral glucocorticoids
• Severe/ failure steroids: Cytotoxic Therapy
- Dr. Ravi Singh @rav7ks
#Cryptogenic #Organizing #Pneumonia #COP #BOOP #pulmonary #ILD #diagnosis #management
The idiopathic interstitial pneumonias (IIPs) comprise a heterogenous group of disorders.
They represent fundamental responses of the lung to injury and do not represent 'diseases' per se.
Idiopathic indicates unknown cause and interstitial pneumonia refers to involvement of the lung parenchyma by varying combinations of fibrosis and inflammation.
IIPs include seven entities listed in the table on the left in order of relative frequency.
These diseases have specific patterns of morphologic findings on HRCT and histology.
Before we call these findings idiopathic or cryptogenic, we should realise, that these patterns are also common findings in collagen vascular diseases (e.g., sclerodermia, rheumatoid arthritis) and drug-related lung diseases.
For instance in patients with rheumatoid arthritis findings of NSIP, UIP, OP and LIP have been reported. #Diagnosis #Radiology #Pulmonary #CTChest #Idiopathic #Interstitial #Pneumonia #IdiopathicInterstitialPneumonia #ILD #Findings #RadiologyAssistant
Ground-glass opacity
Ground-glass opacity (GGO) represents:
- Filling of the alveolar spaces with pus, edema, hemorrhage, inflammation or tumor cells.
- Thickening of the interstitium or alveolar walls below the spatial resolution of the HRCT as seen in fibrosis.
So ground-glass opacification may either be the result of air space disease (filling of the alveoli) or interstitial lung disease (i.e. fibrosis).
The location of the abnormalities in ground glass pattern can be helpfull:
- Upper zone predominance: Respiratory bronchiolitis, PCP.
- Lower zone predominance: UIP, NSIP, DIP.
- Centrilobular distribution: Hypersensitivity pneumonitis, Respiratory bronchiolitis #Diagnosis #Radiology #Pulmonary #CTChest #GGO #GroundGlassOpacity #GroundGlass #Differential #RadiologyAssistant
Basic Interpretation
A structured approach to interpretation of HRCT involves the following questions:
What is the dominant HR-pattern:
reticular
nodular
high attenuation (ground-glass, consolidation)
low attenuation (emphysema, cystic)
Where is it located within the secondary lobule (centrilobular, perilymphatic or random)
Is there an upper versus lower zone or a central versus peripheral predominance
Are there additional findings (pleural fluid, lymphadenopathy, traction bronchiectasis) #Diagnosis #Radiology #Pulmonary #HRCT #CTChest #Interpretation #Algorithm #ILD #RadiologyAssistant
Amiodarone Induced Lung Toxicity Summary
Who?
• 5% of patients
• Dose ≥ 400 mg per day
How? Possible mechanisms:
• Direct cytotoxic lung injury
• Indirect hypersensitivity/immunologic reaction
• Amiodarone half life: 45 days in fatty tissue
Presentation?
• Acutely (hours to days after surgery or angiography)
• Chronically (months to years after starting amiodarone treatment)
• Can rarely present with eosinophilic pneumonia, organizing pneumonia, ARDS, DAH, solitary and multiple pulmonary nodules and masses, and exudative pleural effusions
• Acute toxicity (e.g., acute respiratory distress syndrome) is rare but is associated with high mortality (up to 50%).
• Chronic toxicity (e.g., chronic interstitial pneumonitis, organizing pneumonia) is more common and presents gradually with symptoms including:
1. Non-productive cough
2. Dyspnea
3. Fever
4. Pleuritic chest pain
5. Fatigue and/or weight loss
6. Mortality has been reported as up to 10% in some studies
Characteristic finding is accumulation of lipid-laden 'foamy' macrophages in alveolar spaces.
• Patchy or diffuse infiltrates
• Ground glass opacities
• Reticular + Nodular opacities; traction bronchiectasis
• Most commonly: Interstitial pneumonitis
Diagnosis:
• Exclude other causes like:
- Heart failure
- Infectious pneumonia
- Pulmonary embolism
- Malignancy
- Exclusion of other ILDs
• Presence of foamy macrophages in the BAL
• Improvement in symptoms and radiographic manifestations following withdrawal of amiodarone (with or without glucocorticoid therapy)
• 20% ↓ in DLCO & new ground glass or reticular opacities on chest radiograph and confirmatory findings on HRCT is highly suggestive but not diagnostic of amiodarone-induced pulmonary toxicity.
Labs:
• Nonspecific leukocytosis
• ↑ LDH
• ↑ ESR, CRP
BAL:
• Lymphocytosis
• Neutrophilia
• Eosinophilia
• Normal BAL cellular counts
• Foamy macrophages
Treatment:
• Stopping amiodarone
• Symptomatic patients: Corticosteroids
Prognosis:
• Favorable in case of most common presentation: Interstitial pneumonitis
#Amiodarone #Induced #Lung #Toxicity #Pulmonary #diagnosis #management #Toxicology
Radiographic appearances of pulmonary diseases in HIV/AIDS Patients
• Diffuse Interstitial Infiltrates: Pneumocystis jirovecii, M.tuberculosis, Histoplasma capsulatum, Coccidioides spp., Cryptococcus neoformans, Toxoplasma gondii, Cytomegalovirus, Influenza, Lymphocytic interstitial pneumonitis, Abacavir hypersensitivity; Drug toxicity, Pulmonary edema; ARDS, Metastatic disease, Pulmonary alveolar proteinosis
• Hilar adenopathy: M. tuberculosis, H.capsulatum, Coccidioides spp, Non-Hodgkin's (NHL) or Hodgkin's lymphoma, Mycobacterium avium complex
• Nodular/Masses: M.tuberculosis, C.neoformans, Aspergillus spp., H.capsultaum, Nocardia spp; Actinomyces spp., NHL; Lung cancer, Kaposi sarcoma, Castleman's disease
• Pleural effusion: Parapneumonic effusion; empyema, M.tuberculosis, Lymphoma (NHL, primary effusion lymphoma), Kaposi's Sarcoma
• Focal Consolidation: Pyogenic bacterial pneumonia, M. tuberculosis, Legionella spp., Rhodococcus equi, Bronchoalveolar cancer, Organizing pneumonia
• Cavitary disease: Pyogenic bacterial pneumonia from P.aeruginosa, S. aureus, Enterobacterales; M. tuberculosis, C.neoformans, R.equi, Aspergillus spp., Nocardia spp., Mycobacterium avium complex, P.jirovecii
• Pneumothorax: P.jiroveci
• Normal radiograph: P.jiroveci, M. tuberculosis
Infectious Diseases Fellows Network @ID_fellows
#HIVAIDS #pulmonary #diseases #differential #diagnosis #chestCT #radiology
Bronchiectasis - Summary
What?
• Bronchiectasis is derived from the Greek words bronckos meaning airway and ectasis meaning widening.
• Permanent irreversible dilatation of cartilage containing airways.
Why?
Due to:
• Recurrent infection or inflammation
• Impaired mucociliary clearance
• Chronic inflammation causes irreversible bronchial wall injury → mucous stasis → perpetuating airway obstruction and inflammation
Symptoms:
• Chronic cough
• Hypoxia
• Dyspnea
• Hemoptysis
• Coughing up yellow or green mucus daily
• Fatigue, feeling run-down or tired
• Fevers and/or chills
• Wheezing
• Clubbing of nails
• Loss of weight
• Chest pain/tightness
Most Common Causes:
• Cystic fibrosis
• Aspiration
• Immunodeficiencies
• Connective tissue diseases
• Airway obstruction
• COPD
• Congenital
• Mounier-Kuhn syndrome (congenital tracheobronchomegaly)
• Hypersensitivity: ABPA
• Infection: TB, NTM, PNA
• Primary ciliary dyskinesia
• Young syndrome (bronchiectasis, sinusitis, and obstructive azoospermia and no evidence of cystic fibrosis)
Physical Examination:
• Crackles, rhonchi, scattered wheezing, and inspiratory squeaks on auscultation
• Digital clubbing (2-3% of patients; more frequent in moderate-to-severe cases)
• Cyanosis and plethora with polycythemia from chronic hypoxia
• Wasting and weight loss
• Nasal polyps and signs of chronic sinusitis
• Physical stigmata of cor pulmonale, in advanced disease
Complications:
• Superimposed infection - increase in the peribronchial thickening, presence of air-fluid levels, centrilobular nodules, or consolidation in the adjacent lung parenchyma.
• Pulmonary artery hypertension
• Mosaic attenuation
• Volume loss of the affected lung parenchyma
Diagnosis:
• HRCT (high-resolution chest CT)
• Broncho-arterial ratio (BAR) - BAR >1.0 is abnormal in adults
• Blood tests and sputum cultures (sputum for bacteria and Acid fast)
• Pulmonary function tests: obstructive
• Bronchoscopy
Laboratory:
• ANA, RF, CCP, ANCA
• HIV, Immunoglobulins (Ig M, A, G, E)
• Sweat chloride test, α1-antitrypsin deficiency
Sputum cultures growing uncommon pathogens:
• Pseudomonas aeruginosa
• Aspergillus
• Nontuberculous mycobacteria
Treatment:
• Clearing the airway
• Treating infections
• Preventing exacerbations
#Bronchiectasis #pulmonary #differential #diagnosis
Sjogren's Syndrome Overview
Epidemiology:
• F > M: 9:1
• 5-6th Decades (can be any age)
Autoimmune exocrinopathy multisystemic disease affecting salivary and lacrimal glands:
• Focal lymphocytic sialadenitis
• Keratoconjunctivitis sicca
• Xerostomia
• Symmetric parotid swelling
• Lacrimal swelling
• Skin dryness
• Vaginal dryness
• Primary form: not associated with other diseases
• Secondary form: overlaps with RA, SLE
• Mikulicz syndrome: Prominent parotid and lacrimal glandular enlargement
Sjogren's Syndrome Diagnosis:
• Dry eyes
• Salivary hypofunction
• Antibodies: Anti-Ro/SSA & Anti-La/SSB ab
• Lip (minor salivary gland) biopsy
• Ocular examination
Labs:
• Antibodies:
• Anti-Ro/SSA ab (specific in primary)
• Anti-La/SSB ab
• RF
• Antinuclear antibodies ≥ 1:320
• Cytopenia: 2%
• Hypocomplementemia
• Can cause TYPE IV RTA, TYPE I RTA
Diagnosis:
• LIP (minor salivary gland) biopsy
• Histo: aggregate foci (>50 cells/aggregate) of CD4 T cells, B cells, and plasma cells around salivary ducts
Clinical Features:
HEENT:
• Keratoconjunctivitis sicca
• Xerostomia
• Dental caries
• Parotid swelling
CNS Involvement:
• Central nervous system (CNS): demyelinating disease, myelopathy, cranial nerve neuropathy
• Peripheral nervous system: small-fiber neuropathy, mononeuritis multiplex, peripheral neuropathy, transverse myelitis
• 8%-27% for CNS and peripheral
Thyroid Disease:
• 10%-15%
Pulmonary
• Interstitial Pneumonitis (LIP, UIP, NSIP):
• Lymphocytic interstitial pneumonia
• Usual interstitial pneumonia
• Tracheobronchial disease
• Xerotrachea
• NSIP: Pleural thickening or effusion, INC risk PE, Pulmonary amyloidosis, PAH
Skin:
• Dry skin (xerosis)
• Cutaneous vasculitis: 10%-16%
Liver:
• Autoimmune hepatitis, primary biliary cirrhosis: 3%-20%
Renal:
• Interstitial nephritis
• Distal (Type I) renal tubular acidosis
• NAGMA
• Glomerulonephritis: 5%-6%
• Membranoproliferative GN
• Nephrogenic DI
Hematologic:
• Lymphoma, Cytopenia: 2%
Systemic Vasculitis:
• 7%, Cryoglobulinemia (4%-12%), Raynaud phenomenon (16%)
MSK:
• Arthralgia and Arthritis
Malignancy Risk:
• Non-Hodgkins lymphoma
• B cell lymphoma
• Large B cell lymphoma
• MALT Lymphoma
• Lung Adenocarcinoma
Treatment:
• Symptomatic: artificial tears/artificial saliva/mouth lubricants
• Pharmacologic:
• Severe oral dryness: muscarinic agonists
• Active extraglandular autoimmune disease: Glucocorticoids
• Immunomodulatory/Biologic: 2nd or 3rd line
#Sjogrens #Syndrome #Rheumatology #Diagnosis #Management
Lung Abscess - Diagnosis and Management Summary
Lung Abscess Etiology:
• Necrosis of lung parenchyma by a polymicrobial infection
• Most common cause: Aspiration pneumonia
• DM risk factor for Klebsiella pneumonia
• Chronic alcoholism
• Periodontal disease/poor dentition/gingival disease
• IV substance use
• Esophageal dysmotility
• Depressed consciousness
• Large volume feeding tubes
Lung Abscess Pathophysiology:
1. Inoculum
2. Pneumonitis
3. Tissue necrosis (7-14 days later)
4. Lung abscess
Lung Abscess - Clinical Presentation:
• 80% have fever ≥ 38 degrees
• Chills, night sweats, fatigue, unexplained weight loss
• Late disease: Pleuritic chest pain, hemoptysis, dyspnea, productive cough
• Putrid sputum/sour-tasting sputum
Lung Abscess - Physical Exam:
• Poor dentition
• Gingival crevice disease
• Diminished breath sounds
• Rales
Lung Abscess Diagnosis:
• CBC - Leukocytosis, anemia
• Sputum/Blood cultures
• CXR (non-diagnostic in early disease)
• CXR upright or lat decubitus - air fluid level seen
• Chest CT
Lung Abscess Management:
• Antibiotics:
- IV Ampicillin-sulbactam
- Piperacillin/Tazobactam - pseudomonas
- Carbapenems
- IV Clindamycin in case of PCN allergy
- Switch to oral antibiotics once stable
- 10-15% require lobectomy or pneumonectomy if fail antibiotics
• Consider Surgical Intervention:
- Fevers > 10 days
- Hemoptysis
- Cavitation > 6 cm
- Neoplasm, or hemorrhage
• Treatment duration:
- 3 weeks or continue antibiotic treatment until the chest radiograph shows a small, stable residual lesion or is clear. This generally requires several months of treatment
• Delayed response: Evaluate for foreign body, cancer, or bronchial stenosis
Lung Abscess Pathogens:
• Aerobic Bacteria:
- Staph. Aureus
- Legionella
- H. Influenza
- E. Coli
- P. Aeruginosa
- Strep. Pyogenes
- K. Pneumonia
• Anaerobic Bacteria:
- Bacteroides
- Fusobacterium
- Peptostreptococcus
- Prevotella
• Fungal:
- Aspergillosis
- Blastomyces
- Dermatitidis
- Cryptococcus
- Coccidioides
• Parasite:
- Entamoeba histolytica
- Paragonimus westermani
• Other: TB, M. avium, M. kansii
Lung Abscess Differential Diagnosis:
• Vasculitis (e.g., Granulomatosis with polyangiitis)
• Malignancy
• Aspirated foreign body
• Bronchostenosis
#Lung #Abscess #pulmonary #differential #causes #management #treatment #diagnosis
Autoimmune Myositis - Differential Diagnosis Framework
Inflammatory Myopathies: Commonly symmetric proximal muscle weakness, no substantial muscle pain or tenderness; serum creatine kinase ↑
Muscle Tenderness → Consider infectious, thyroid, drug-induced myopathies
Diagnostic Tests:
• Measure CK
• Aldolase levels
• EMG
DERMATOMYOSITIS:
• Subacute
• Proximal muscle (symmetric)
• CK > 50 ULN
Antibodies:
• Anti-MDA5 (RP-ILD), dermato-rheumatologic symptoms
• Anti-CADM-140 (amyopathic dermatomyositis)
• Anti-Mi2 (skin lesions)
• Anti-TIF1 (cancer-associated adult dermatomyositis)
• Anti-NXP2 (ANA present 80% of the time)
Dermatomyositis Symptoms:
• Gottron rash (Gottron papules and Gottron sign)
• Shawl sign
• Heliotrope
• Interstitial lung disease
Malignancy Risk: Ovarian, lung, pancreas, stomach, colon, and lymphoma
POLYMYOSITIS:
• Progressive, symmetric, proximal muscle weakness
• CK > 50 ULN
Antibodies:
• Anti-Synthetase Ab
• Anti-Jo-1 antibodies (ANA present 80% of the time)
Polymyositis Symptoms:
• Muscle pain and tenderness
• Muscle atrophy
• Weakness of respiratory muscles
• Interstitial lung disease
• Dysphagia, dysmotility, and increased risk of aspiration pneumonia
• Antisynthetase syndrome (also seen in association with dermatomyositis)
Malignancy Risk: Ovarian, lung, pancreas, stomach, colon, and lymphoma
Overlap Syndrome: Fever, joint pain and Raynaud phenomenon
INCLUSION BODY MYOSITIS:
• M > F
• Age > 50
• Misdiagnosed as: Polymyositis or ALS
• Very slow onset and pattern of muscle involvement/Early weakness
• History of preexisting weakness averaging 5 years
• Proximal + distal muscle involvement
• Symmetric, but asymmetry may occur
• Frequent falls due to quadriceps muscle weakness
PE: Muscle weakness + atrophy- Hip flexors, quadriceps, finger flexors, and forearm flexors
Labs:
• Serum creatine kinase levels are elevated < PM and DM
• CPK 10X ULN
• + Camptocormia (bending forward of the spine) or head drop
• ANA < 20 % IBM
• Anti-cytoplasmic 5'-nucleotidase 1A (Anti cN-1A)
50% Cricopharyngeal muscle involvement -> dysphagia and increased risk of aspiration > 50% of pts
ANTI-SYNTHETASE SYNDROME:
• Fever
• Interstitial lung disease
• Myositis
• Raynaud phenomenon
• Nonerosive arthritis
• Mechanic's hands
Autoantibodies:
• Anti-aminoacyl-tRNA synthetase enzymes
• Anti-Jo-1 antibodies
Symptoms:
• Pericarditis/Pericardial tamponade
• Conduction system abnormalities/Arrhythmias
• Dysphagia, dysmotility, and increased risk of aspiration pneumonia (esophagus upper 2/3 striated muscle)
NECROTIZING AUTOIMMUNE MYOPATHY:
• Proximal muscle (symmetric)
• Prominent myonecrosis on biopsy
• Severe, rapidly progressive weakness
• Very high serum CK levels 50X ULN
• Myocyte necrosis and regeneration without significant inflammation
• Extramuscular manifestations rare
• Possible paraneoplastic syndrome
• If myopathy improves within 4 to 6 weeks after discontinuation of statins - probably caused by toxic effects of the drug
Antibodies:
• Signal recognition particles (SRP's)
• 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase
ANTI-MITOCHONDRIAL MYOPATHY:
• Chronic skeletal muscle disease/muscular atrophy
• Chronic progressive respiratory muscle weakness
• Severe cardiac involvement: (20-30%) arrhythmias, cardiomyopathy, and myocarditis
Other Diseases:
• PBC, autoimmune hepatitis, Psoriasis, Hashimoto's
Antibodies:
• Anti-mitochondrial antibodies
#Autoimmune #Myositis #Myopathy #rheumatology #differential #diagnosis
