Inguinal hernias

1. ACUTE RHEUMATIC FEVER
AND BRONCHIESTASIS
Group A

2. ACUTE RHEUMATIC
FEVER

3. • Acute rheumatic fever is a multisystem autoimmune disease affecting the
heart and extra- cardiac sites (joints, brain, skin and others) that typically
develops after an episode of Group A beta hemolytic streptococcal
pharyngitis or infection

4. EPIDEMIOLOGY
• Mainly a disease of children aged 5 – 14 years.
• Initial episodes become less common in older adolescents and young adults and are rare in
persons aged >30 years.
• By contrast, recurrent episodes of ARF remain relatively common in adolescents and young
adults.
• This pattern contrasts with the prevalence of Rheumatic Heart Disease, which peaks between 25
and 40 years.
• Rheumatic fever mostly affects children and adolescents in low- and middle-income countries.
• People who live in overcrowded and poor conditions are at greatest risk of developing the disease.

5. ETIOLOGY
• The aetiologic agents are group A beta hemolytic streptococci (GABHS).
• The disease develops 2-3 weeks after streptococcal pharyngitis.
• There is molecular mimicry between streptococcal M proteins and various
proteins in the heart, brain and joints.

6. This leads to cross reactivity between antibodies produced against M proteins
and proteins in myocardial and heart valves as well as other tissues such as
joints, skin and nerves.

7. PATHOPHYSIOLOGY
• GABS infections usually causes pharyngitis, but also rarely with infections at
other sites such as skin . Antigen target in strep pyogenes is the M protein.
This leads to local inflammation to the throat mucosa. Neutrophil
phagocytosis and Antigen presentation to T cells (CD4+) in local Lymph
nodes. Antibody production against M protein; targeted to pharyngeal
antigen(97%) and other tissues due to molecular mimicry (3%)Other tissues
include ; Heart (50%-60%), CNS, joints and skin.

8. 1. Heart
• Most common cause of acquired valvular heart disease in underdeveloped
countries is rheumatic fever. These antibodies cross react with human tissues
because of the antigenic similarity between M protein of strep pyogenes.
The tissues affected are the Pericardium (fibrinous pericarditis), myocardium
( Aschoff body) and endocardium involved. The antibody targeted mimicry
proteins include; Tropomyosin, myosin, actin, troponin

9. • The inflammatory process lead to formation of vegetations "fibrinous
clots" along the lines of valve closure ( Verrucous endocarditis) and
thickening of the chordae tendinae and increased risk to sub acute
bacterial endocarditis.
• The characteristic lesion in the myocardium is a focal inflammatory foci
known aschoff bodies. A focus of fibrinoid necrosis surrounded by a
collection of lymphocytes, plasma cells macrophages, modified histiocytes
known as Anitschow cells , they may becomes multinucleated forming
Aschoff giant cells

11. • Due to cross-reactivity between streptococcal antigens and basal ganglia protein
• Occurs in 10% of the patients.
• More common in females.
• Usually a late manifestation. Latent period varies. May occur 3 months after an episode of RF, or even several
decades later.
• Emotional disturbances, chorea, speech abnormalities
• Patient should be given prophylaxis to prevent further attacks.
• Spontaneous resolution may occur in a few months.
• 25% develop RHD.
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2. Sydenham Chorea

12. • Early feature
• Associated with high titers of Strep antibodies
• Due to cross-reactivity between streptococcal antigens and vimentin.
• Present in 75% of patients
• Characterized by acute, painful, asymmetric and migratory inflammation of large
joints (knees, elbows, wrist ,ankle)
• Does not affect small joints of the hands, feet
• Joints are affected in quick succession
• Presents as tender, warm, swollen joints
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3. Migratory Polyarthritis

13. CLINICAL FEATURES
• Symptoms in all age groups typically begin 2 to 3 weeks after streptococcal infection
• Migratory polyarthritis - one large joint after another becomes painful and swollen for a
period of days, followed by spontaneous resolution with no residual disability.
• Carditis including pericardial friction rubs and arrhythmias
• Subcutaneous nodules and erythema marginatum skin rashes
• Sydenham chorea, a neurologic disorder characterized by involuntary purposeless, rapid
movements(also called St Vitus dance)
• Fever
• Arthralgias
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14. INVESTIGATIONS
1. Evidence of preceding streptococcal infection
• Throat swab culture is positive for group A beta hemolytic streptococci.
• ASOT- Rise in antistreptolysin O antibody titres or levels of >200 U in adults and >300 U in children.
2. Evidence of a systemic illness
• Full hemogram- Neutrophilic leukocytosis
• Acute Phase Reactants(ESR and CRP)- Increased
3. Evidence of carditis
• Electrocardiography- Prolonged QRS interval and AV block(prolonged PR interval)
• Chest X-ray- Cardiomegaly and pulmonary congestion
• Echocardiography- Cardiac dilatation and valvular abnormalities such as mitral regurgitation.
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15. DIAGNOSIS
Diagnosis is mainly made using the revised Jones criteria that is based upon:
• 2 or more Major manifestations
• One Major and two or more Minor manifestations
• along with serological evidence of preceding streptococcal infection
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16. Major manifestations Minor manifestations
• Carditis
• Migratory polyarthritis
• Sydenham chorea
• Erythema marginatum
• Subcutaneous nodules
• Fever
• Arthralgia
• Leukocytosis
• First degree AV block
• Raised ESR or CRP
• Previous rheumatic fever

17. TREATMENT
• Involves four steps;
- Step I – primary prevention
- Step II – anti-inflammatory treatment
- Step III – supportive management
- Step IV – Secondary prevention

18. Step I: Primary Prevention Of Rheumatic
Fever
• Penicillin G – IM (Once)
• Penicillin V – Oral (Daily - 10 days)
• Erythromycin – Oral (Daily – 10 days) For individuals allergic to penicillin.

19. Step II: Anti – inflammatory treatment
• Arthritis – Aspirin
• Carditis - Prednisolone

20. Step III: Supportive Management And
Management of Complications
• Bed rest
• Treatment of congestive heart failure – Diuretics, ACEI
• Treatment of Chorea – diazepam or haloperidol
• Rest to joints and supportive splinting.

21. Step IV: Secondary Prevention of Rheumatic
Fever
• Penicillin G
• Penicillin V
• Sulfadiazine
• Erythromycin – individuals allergic to penicillin and sulfadiazine.

23. BRONCHIECTASIS

24. • Destruction of smooth muscle and elastic tissue by inflammation stemming
from persistent or severe infections leads to permanent dilation of bronchi
and bronchioles.

25. ETIOLOGY
• Congenital or hereditary conditions that predispose to chronic infections: cystic fibrosis,
immunodeficiency states, primary ciliary dyskinesia and kartagener syndrome.
• Infections: as a complication of severe necrotizing pneumonia caused by bacteria, viruses or fungi.
• Bronchial obstruction: tumor, foreign body, or mucus impaction; bronchiectasis is localized to the
obstructed lung segment.
• Autoimmune: rheumatoid arthritis, SLE, inflammatory bowel disease and chronic rejection after
lung transplant and chronic graft versus host disease after hematopoietic stem cell transplantation.
• Idiopathic up to 50% of the cases: appears to be dysfunctional host immunity to infectious agents
leading to chronic inflammation.

26. PATHOPHYSIOLOGY
• There’s infection with chronic or recurrent bouts causing destruction
of supporting smooth muscle and elastic tissue
• The bronchi become markedly dilated while the bronchioles become
progressively obliterated as a result of fibrosis(bronchiolitis
obliterans)
• Cystic fibrosis - primary defect in ion transport result in thick
viscous secretions that perturb mucocilliary clearance and lead to
airway obstruction

27. • Primary ciliary dyskinesia - mutations result in ciliary dysfunction
due to defects in ciliary motor proteins again preventing mucociliary
clearance setting the stage for recurrent infections that lead to
bronchiectasis
• Kartagener syndrome - ciliary function is necessary during
embryogenesis to ensure proper rotation of the developing organs
in the chest and abdomen. In it’s absence patients with primary
ciliary dyskinesia have situs inversus or partial lateralising
abnormality associated with bronchiectasis and sinusitis

28. • Allergic bronchopulmonary Aspergillosis - it is a hyperimmune
response to Aspergillus fumigatus. Sensitization to aspergillus
leads to activation of Th2 helper T cells which release
cytokines that recruit eosinophils and other leucocytes. There
are high serum IgE levels and intense airway inflammation
with eosinophils and formation of mucus plugs which play a
primary role in the development of bronchiectasis

29. • The lower lobes are usually bilaterally affected and is
more severe in the distal bronchi and bronchioles
• There is dilation of bronchi and bronchioles even up to 4
times the normal size and are filled with mucopurulent
secretions.

30. CLINICAL FEATURES
• Persistent chronic cough
• Production of purulent tenacious sputum
• Pleuritic pain when the pleura is involved
• Dyspnea and orthopnea in severe cases
• Halitosis
• On physical examination, there is crackles and wheezing on auscultation and finger
clubbing.
• Pulmonary function tests show FEVC1/FVC less that 0.7 to indicate and obstructive
disease.

31. DIAGNOSIS
• It is based on presentation with a persistent chronic cough and sputum
production accompanied by consistent radiographic features.
Chest radiographs:
• Lacks sensitivity
• Presence of ‘tram trucks’ indicating dilated airways is consistent with
bronchiectasis.

32. Chest CT:
• Its more specific
• Imaging modality of choice for confirming diagnosis of bronchiectasis
Findings include:
• Airway dilation which can be detected as parallel ‘tram trucks’ or as the ‘Signet-ring
sign’ (which refers to the appearance in cross section of a dilated air-filled bronchus
that is continuous with the smaller nodular opacity of a pulmonary artery.)
• Lack of bronchial tapering
INVESTIGATIONS

33. • Bronchial wall thickening in dilated airways
• Inspissated secretions (e.g. the ‘tree-in-bud’ pattern) i.e. when linear
branched markings are noted when airway contain mucopurulent plugs.
• Airway visibility within 1 cm of a costal pleural surface or touching the
mediastinal pleura
• Cysts emanating from bronchial wall (esp. pronounced in Cystic
bronchiectasis it appears like grapes).

34. LABORATORY INVESTIGATION
• Sputum – Mainly for culture for bacteria, mycobacteria and fungi.
• Complete Blood Count - Typical findings are nonspecific and include anemia and
an elevated white blood cell count with an increased percentage of neutrophils.
Polycythemia secondary to hypoxia may be observed in advanced cases.
• Pulmonary Function Test - It is used for functional assessment of impairment
due to bronchiectasis. Spirometry before and after the administration of a
bronchodilator is satisfactory in most patients. Obstructive impairment (i.e.,
reduced or normal FVC, low FEV1, and low FEV1/FVC) is the most frequent
finding, but a very low FVC can also be seen in advanced disease in which much
of the lung has been destroyed.

35. • A posterior-anterior chest radiograph with walls of
airways dilated and thickened (arrow) in the right
upper lobe as seen in allergic bronchopulmonary
aspergillosis. In the left upper lobe are airways filled
with mucus and cellular debris
• Lateral chest radiograph demonstrating ring shadows
of cystic bronchiectasis (arrow).

37. Tram truck sign
Cystic
bronchiectasis

38. Management Of Acute Exacerbations
Approach
• Provide supportive treatment and oxygen therapy as needed
• Optimize mucoactive agents
• Optimize airway clearance techniques
• Obtain a new sputum culture and start empiric antibiotic therapy, based on
the most recent sputum culture
• Tailor antibiotics to the most recent sputum culture once available

39. Monitoring and disposition
• Outpatient treatment for hemodynamically stable patients with to moderate
symptoms
• Consider inpatient treatment in cases such as : clinical deterioration or no
clinical improvement following completion of an oral antibiotic regimen,
sepsis, severe pneumonia, massive hemoptysis>10ml in 24hrs, new isolation
of P, aeruginosa.

40. Common Empiric Antibiotic Regimens For
Bronchiectasis Exacerbation
• No previous culture data available consider fluoroquinolones
• ciprofloxacin
• Levofloxacin
• Colonization with beta lactamase positive organisms consider amoxicillin/clavulanic acid
or doxycycline
• Colonization with P. Aeruginosa consider ciprofloxacin
• Colonization with sensitive pathogens consider amoxicillin or doxycycline or
clarithromycin
• Colonization with MRSA consider vancomycin or linezoid

41. Long Term Management
• The goal is to stop or delay disease progression, reduce exacerbation frequency, achieve
symptom control and improve patient’s quality of life.
• Lifestyle changes like regular exercise and smoking cessation
• Educate patient on airway clearance techniques - bronchopulmonary hygiene and chest
physiotherapy, pulmonary rehabilitation.
• Administer vaccination i.e.. influenza vaccine and pneumococcal vaccine
• Consider treatment with mucoactive agents e.g.. mannitol and hypertonic saline,
bronchodilators e.g.. SABA and LABA or corticosteroids though not routinely
recommended, if airway clearance is difficult.
• Provide specific treatment for the underlying cause if unidentified

42. • Follow ups ever 6-12 months to identify disease progression - sputum
culture and spirometry
• Consider long term antibiotic therapy for bronchiectasis with >3
exacerbations per year

43. Long Term Antibiotic Therapy For
Bronchiectasis
• If no colonization with P. aeruginosa consider oral macrolides e.g..
azithromycin or inhaled antibiotics e.g. tobramycin
• If colonization with P. aeruginosa consider Tobramycin, aztreonam for
growth suppression.
• If Nontuberculous mycobacteria consider Macrolide plus rifampicin plus
ethambutol for at least one year

44. Invasive Procedures
• Surgical resection of bronchiectasis lung or lobectomy indicated in
pulmonary hemorrhage, inviable bronchus and poor control of symptoms
despite optimal medical therapy in unilateral bronchiectasis with well
localized disease.
• Pulmonary artery embolization : indicated in pulmonary hemorrhage
• Lung transplantation in severe disease or rapid disease progression.

45. LOCAL COMPLICATIONS SYSTEMIC COMPLICATIONS
Recurrent pneumonia Septicemia
Lung abscess Secondary amyloidosis with nephrotic syndrome
Empyema
Hemoptysis
Pulmonary hypertension with cor-pulmonale
Congestive cardiac failure
Respiratory failure