SYSTEMIC SCLEROSIS - Diagnostic criteria

ACR/EULAR classification criteria for systemic sclerosis:

A total score of 9 or more is required for classification as systemic sclerosis.

1. Skin thickening of the fingers:

   - Proximal to the metacarpophalangeal joints (9 points).

2. Fingertip lesions:

   - Digital tip ulcers (2 points).

   - Fingertip pitting scars (3 points).

3. Telangiectasia (2 points).

4. Abnormal nailfold capillaries (2 points).

5. Pulmonary involvement:

   - Interstitial lung disease or pulmonary arterial hypertension (2 points).

6. Raynaud's phenomenon (3 points).

7. SSc-specific autoantibodies:

   - Anti-centromere, anti-topoisomerase I (Scl-70), or anti-RNA polymerase III (3 points).

SLE

SLE (American College of Rheumatology criteria )

1)Malar rash

2)Discoid rash

3)Photosensitivity skin rash

4)Oral or nasopharyngeal ulceration

5)Non erosive arthritis involving ≥ 2 peripheral joints

6)Serositis (pleuritis or pericarditis)

7)Renal disorder (persistent proteinuria or cellular casts)

8)Neurologic disorder (unexplained seizures or psychosis)

9)Hematologic disorder (hemolytic anemia, leukopenia, lymphopenia,or thrombocytopenia)

10)Immunologic disorder (positive LE cell, anti-DNA antibody, anti-Sm antibody, false-positive syphilis serology)

11)Elevated antinuclear antibodies

*Minimum of 4 criteria required

SJÖGREN SYNDROME

 sicca symptoms are mandatory

 supportive evidence including ocular signs (positive Schirmer test testing reduced tear formation, rose bengal score > 3 for staining of conjunction and cornea)

 typical histologic appearances salivary gland biopsy

antibodies to Ro (SS-A) or La (SS-B) or

 reduced salivary flow.

RHEUMATOID ARTHRITIS - Diagnostic criteria

American Rheumatism Association revised criteria

1)Morning stiffness (lasting at least 1 hr)

2)Arthritis (soft tissue swelling or fluid) of 3 or more joints (PIP, MCP,wrist, elbow, knee, ankle, MTP joints)

3)Arthritis of hand joints (swelling of at least 1 wrist, MCP, or PIP joint)

4)Symmetrical arthritis (i.e., simultaneous arthritis of the same joints on both sides of the body)

5)Rheumatoid nodules

6)Serum rheumatoid factor positivity (at a level such that < 5% of normal controls are positive)

7)Radiographic hand or wrist changes typical of rheumatoid arthritis

At least 4 criteria for a minimum of 6 weeks

Sjogren's syndrome

Classification criteria for sjogren's syndrome 

1) Labial salivary gland with focal lymphocytic sialadenitis and focus score of > 1 foci/4mm²    

        3 points

2) Anti-SS-A/Ro positive

        3 points

3) Ocular Staining Score 5 (or van Bijsterveld score > 4) in at least one eye

         1 point

4) Schirmer's test < 5 mm/5 minutes in at least one eye

         1 point

5) Unstimulated whole saliva flow rate < 0.1 ml/minute

          1 point

A score of 4 classifies a patient who meets the inclusion criteria:

⁃ ocular and/or oral dryness or suspicion of SjS according to EULAR SjS Disease Activity Index (ESSDAI)

and does not have any of the exclusion criteria:

⁃ history of head and neck radiation, active HCV infection, AIDS, sarcoidosis, amyloidosis, graft-versus-host disease, IgG4-related disease.

* ACR/EULAR classification criteria

AGGRAVATING FACTORS OF CARDINAL SYMPTOMS- MNEMONICS:

COUGH:

PDFCE 

Pollution, Pollen, Posture

Drugs, Diurnal, Dry air

Food

Cold weather, Common cold

Exercise

SPUTUM

 "DRIVE DUST"

 

D – Dust exposure

R – Respiratory infections

I – Irritants (e.g., smoke, fumes)

V – Viral infections

E – Exercise or exertion

D – Dry air

U – Upper respiratory tract conditions (e.g., postnasal drip)

S – Smoking

T – Temperature changes (cold air or sudden temperature drops)

Breathlessness: 

 "BREATHE HARD"

 

B – Body position (e.g., lying flat in orthopnea)

R – Respiratory infections

E – Exercise or exertion

A – Anxiety or emotional stress

T – Temperature extremes (cold or heat)

H – Hypoxia or high altitude

E – Environmental triggers (allergens, pollutants)

H – Heart failure or fluid overload

A – Anemia

R – Reflux (gastroesophageal reflux disease)

D – Drugs (e.g., beta-blockers, 

WHEEZE:

“BREATHE WHEEZE”

B – Bronchial irritants (smoke, pollution)

R – Respiratory infections (viral or bacterial)

E – Exercise

A – Allergens (pollen, dust mites, animal dander)

T – Temperature changes (cold air)

H – Hormonal changes (pregnancy, menstruation)

E – Emotions (stress, anxiety)

W – Workplace irritants (occupational exposures)

H – Household triggers (perfumes, cleaning agents)

E – Environmental changes (high altitude)

E – Exposure to drugs (NSAIDs, beta-blockers)

Z – Zzz… sleep (nocturnal asthma triggers)

E – Eating (GERD-induced wheeze)

CHEST PAIN:

“CHEST PAINS”

C – Coughing or deep breathing (pleuritic pain)

H – Heat or cold exposure (vasospastic angina)

E – Emotion or stress (psychogenic pain, angina)

S – Swallowing (esophageal causes like GERD or spasm)

T – Trauma or movement (musculoskeletal pain, costochondritis)

P – Physical exertion (angina, cardiac ischemia)

A – Alcohol or large meals (GERD or esophageal spasm)

I – Infections (pleuritis, pericarditis, pneumonia)

N – Nighttime (GERD-related pain due to supine position)

S – Specific postures (pericarditis, GERD, musculoskeletal pain)

HEMOPTYSIS:

“COUGH BLOOD”

C – Coughing (increases airway pressure, ruptures small vessels)

O – Overexertion (exacerbates vascular stress)

U – Upper respiratory tract infections

G – GERD or vomiting (irritation of airways)

H – High altitude (pulmonary hypertension)

B – Bronchial infections (bronchitis, bronchiectasis)

L – Lung malignancy (coughing or irritation)

O – Operations (post-procedural bleeding, e.g., bronchoscopy)

O – Oral anticoagulants (worsen bleeding)

D – Deep breaths or physical strain (ruptures fragile capillaries)

Aggravating and relieving factors for cough , breathlessness, chest pain:

 1. COUGH

Aggravating factors:

Pollution, Pollen, Posture

Drugs, Diurnal, Dry air

Cold weather, Common cold

Food, Exercise

Relieving Factors:

Using cough suppressants or expectorants

Drinking warm fluids, Humidified air or steam inhalation

Avoiding triggers, Rest

2. BREATHLESSNESS:

Aggravating factors:

Exercise, 

Pollens,dust,smoke,cold air

Drugs- Aspirin,NSAIDs can aggravate breathlessness in asthmatics

Orthopnea- LV failure, large pleural effusion,massive ascites, morbid obesity and any severe lung disease.

Platypnea- ASD, patent foramen ovale, large intrapulmonary shunt,hepatopulmonary syndrome

Trepopnea- Due to large unilateral lung disease(patient prefers healthy lung down), dilated cardiomyopathy (pt prefers right side down)

Tumors compressing major airways/blood vessels 

Relieving Factors:

Rest or reducing physical activity

Oxygen therapy 

Bronchodilators 

Calm breathing techniques 

Elevating the head or upper body in severe cases

3. CHEST PAIN

Aggravating Factors:

Deep breathing, coughing, sneezing (if pleuritic)

Physical exertion (if cardiac in nature)

Stress or anxiety

Movement or pressure on the chest area

acid reflux

Relieving Factors:

Rest 

Pain medication

Positioning (e.g., lying on one side for pleuritic pain)

Relaxation or stress-reduction techniques

Oxygen therapy

Aggravating and relieving factors for pedal edema

 Aggravating and relieving factors for pedal edema

Aggravating factor

Prolonged immobility

Dietary- excess fluid 

Reliving factor

Compression 

Limb elevation

Diet- fluid and sodium restriction 

Reference: Hutchison and macleod

Causes of orthopnoea

 CAUSES OF ORTHOPNEA :

1. Left ventricular failure.

2. Asthma

3. COPD

4. Bilateral diaphragmatic paralysis in the absence of heart disease.

Ref : Fraser 4th edition page no. 388

Breathlessness aggravating and relieving factors

 Breathlessness aggravated by exercise - exercise induced asthma

Aggravated by Pollens,dust,smoke,cold air, drugs, animals - Asthma 

Drugs- Aspirin,NSAIDs can aggravate breathlessness in asthmatics

Aggravated by lying down- orthopnea(relieved by sitting)- LV failure, large pleural effusion,massive ascites, morbid obesity and any severe lung disease.

Aggravated by sitting up- platypnea (relieved by lying down)- ASD, patent foramen ovale, large intrapulmonary shunt,hepatopulmonary syndrome 

Aggravated by lying on oneside - trepopnea

Due to large unilateral lung disease(patient prefers healthy lung down), dilated cardiomyopathy (pt prefers right side down)

Tumors compressing major airways/blood vessels 

Referrence - Macleod's 13 th edition 

Respiratory system page no-142

Monkey pox -etiology and drug of choice

Monkey pox 

 

Etiology :Poxviruses are a family of double-stranded DNA viruses whose genomic structure is generally conserved across subfamilies, genera, and species.

Most poxviruses that infect humans are spread through contact, not  by respiratory route 

Patient usually presents with nodular or vesicopustular lesions ,fever ,followed by rash,centrifugal distributionprominet in palms and soles

 

 Treatment :mainly supportive and prevent secondary bacterial infection

Recently u.s FDA approved the anti viral drug Tecovirmat (Tpoxx) has been used.

Brincidofovir (tembexa)also approved by FDA

Ref : Harrison principal of internal medicine 

Indications of Aerobika

 Indications of AEROBIKA

COPD

Cystic fibrosis

Bronchiectasis

Bronchial asthma

Bronchiolitis obliterans

Relative contraindications to Aerobika

Untreated pneumothorax

Intracranial pressure > 20mm Hg

Active haemoptysis

Recent trauma or surgery to skull, face, mouth, or oesophagus

Patient with acute asthma attack or acute worsening of Chronic Obstructive Pulmonary Disease (COPD) unable to tolerate increased work of breathing

Acute sinusitis or epistaxis

Tympanic membrane rupture or other known or suspected inner ear pathology

Nausea

Source- aerobika user guide

https://www.aerobika.us/wp-content/uploads/2018/10/patient-user-guide-en.pdf? 1

Chromogranin A

 Chromogranin A (CgA) is a constituent of neuroendocrine vesicles and catecholamine storage vesicles . Neuroendocrine cancer , like small cell carcinoma of lung has been found to have CgA secretion and hence used as a tumor marker . It has been used as a marker for disease progression and can also be demonstrated in immunohistochemistry of Neuroendocrine tumors .

 Indications for Noninvasive Mechanical Ventilation (NIV) in COPD

At least one of the following:

1) Respiratory acidosis (PaCO2 > 6.0 kPa or 45mmHg and arterial pH < 7.35)

2) Severe dyspnea with clinical signs suggestive of respiratory muscle fatigue, increased work of

breathing, or both, such as use of respiratory accessory muscles, paradoxical motion of the

abdomen, or retraction of the intercostal spaces

3) Persistent hypoxemia despite supplemental oxygen therapy

Reference : GOLD 2024

MECHANISM OF NORMAL BREATH SOUNDS

Normal breath sounds originate from the larynx. When the sound leaves the larynx it travels down the trachea and then divides when the airway divides. Some sound must be transmitted through the lung parenchyma but most travels down the airway. Eventually the sound travels along airways of different lengths and therefore becomes out of phase. Next it arrives in the respiratory bronchioles and alveoli and then gets transmitted through the chest wall to your stethoscope. The fat layer filters out much of the high frequency sound (above 4 kHz). The resulting sounds are much softer (because the sound has effectively been diluted throughout the whole of the lungs). There is no gap between inspiration and expiration (because all of the sound has become out of phase and therefore filled in the gap. Finally, the first third of expiration is now the only part that is audible because the latter two-thirds are much quieter.

REFERENCE : CHAMBERLAIN'S symptoms and signs of clinical medicine, 13th edition

 ILO CLASSIFICATION TO DESCRIBE PNEUMOCONIOTIC OPACITIES

ILO TERM                  OPACITY WIDTH                              OPACITY TYPE

P                                   < 1.5mm                                               Small rounded

Q                                  1.5 - 3 mm                                            Small rounded

R                                   > 3 - 10 mm                                         Small rounded

S                                   < 1.5mm                                               Linear/ Irregular

T                                   1.5 - 3 mm                                            Linear/ Irregular

U                                   > 3 - 10 mm                                         Linear/ Irregular

A                                   > 10 - 50 mm                                        Large

B                                   > 50 mm - RUZ                                    Large

C                                    > RUZ                                                  Large

Cardinal symptoms

 1. COUGH

Aggravating factors:

Pollution, Pollen, Posture

Drugs, Diurnal, Dry air

Food

Cold weather, Common cold

Exercise

Relieving Factors:

Using cough suppressants or expectorants

Drinking warm fluids 

Humidified air or steam inhalation

Avoiding triggers 

Rest

2. SPUTUM

Aggravating Factors:

Respiratory infections (e.g., bronchitis, pneumonia)

Exposure to cold air or smoke

Allergic reactions

COPD, asthma

Physical activity 

Relieving Factors:

Hydration 

Expectorants 

steam inhalation

Deep breathing exercises

3. HEMOPTYSIS 

Aggravating Factors:

Trauma or injury to the respiratory tract

Relieving Factors:

Treating the underlying condition 

Cough suppressants 

Oxygen therapy 

Positioning the person to reduce coughing or to prevent aspiration

4. CHEST PAIN

Aggravating Factors:

Deep breathing, coughing, sneezing (if pleuritic)

Physical exertion (if cardiac in nature)

Stress or anxiety

Movement or pressure on the chest area

acid reflux

Relieving Factors:

Rest 

Pain medication

Positioning (e.g., lying on one side for pleuritic pain)

Relaxation or stress-reduction techniques

Oxygen therapy

5. BREATHLESSNESS

Aggravating Factors:

Physical exertion or exercise

Exposure to cold air or allergens

Respiratory infections

Anxiety or panic attacks

Relieving Factors:

Rest or reducing physical activity

Oxygen therapy 

Bronchodilators 

Calm breathing techniques 

Elevating the head or upper body in severe cases

6. WHEEZE

Aggravating Factors:

Exposure to allergens or irritants (e.g., smoke, dust, pollen)

Respiratory infections 

Exercise or physical activity 

Cold air or sudden weather changes

Anxiety or panic attacks

Relieving Factors:

Bronchodilators 

Avoidance of triggers

Breathing exercises 

Inhaling steam

Rest and calm environment