Sunday, May 29, 2022

40 year old woman with multiple joint pains

 A 40 year old woman, working as a tailor presented to our outpatient unit with the complaints of

Multiple joint pains since the past 7 years.

Present Illness:

A 40 year old woman was apparently asymptomatic 7 years back after which she started experiencing  multiple joint pains. She reported that she first experienced right proximal interphalangeal joint pain 7 years back while she was working on the sewing machine. After 2 weeks she started to experience left proximal interphalangeal joint pain. Over the last 7 years, she started experiencing multiple joint pains - bilatered meta-carpophalangeal joint,  bilateral elbow joint, bilateral wrist joint, cervival joint, bilateral knee joint, bilateral ankle joint pains. She reported early morning stiffness lasting for more than an hour which would be relieved on physical activity. She would experience these pains intermittently and would often be accompanied by swelling of the joint and would be relieved on taking pain medications. She reported that she developed bilateral little finger deformity 1 year back.

She however gave no history of fever,  oral ulcers, rash, dryness of the skin, hair loss, any development of rash on exposure to sunlight, discouloration of skin on exposure to cold.

No other constitutional symptoms like fever, fatigue, weight loss.

Past History: 

No other significant past history

Personal History:

She is happily married and a mother of 2 children. She has a good appetite, normal bowel and bladder movements.

Family History:

No significant family history 

Provisional Diagnosis: 

Chronic, multiple, symmetrical joint involvement, involvement of PIP & DIP joints - ? Rheumatoid Arthritis

Examination:

Pulse Rate: 75 beats per minute

Blood Pressure: 120/70mmhg

Respiratory Rate: 22 cycles per minute

Temperature: 98.6 F

No pallor, icterus, cyanosis, clubbing, lymphadenopathy, edema 

General Examination:

Hair:

Normal hair distribution, normal texture, colour

Eyes:

No conjunctival injection, no erythema, no corneal lesions

Oral Cavity:

No mucosal ulcers 

Nails:

No nail pitting, onycholysis, onychodystrophy

Skin:

No rash , ulcers over the skin, scaly lesions, dryness of skin, thickening of skin, no rash on sun exposed areas of the skin, no subcutaneous nodules

Spine:

No spinal deformity

Musculoskeletal System Examination: 

Gait: Normal


Musculoskeletal system:

Upper limbs:

Boutenniere' or button hole deformity of the right little finger &

Swan neck deformity of the left little finger:





Prayer sign:



No tenderness or erythematous noted on examination of the joints








Restriction of joint motion noted only in the right PIP  joint and left DIP joint
No restriction of other joint movements







Shoulder joint examination: No swelling or erythema of shoulder joints
No restriction of joint movement
Apprehension test negative - For looking for glenohumeral instability 



















No tenderness of knee & ankle joints
No joint restriction






Absent Achilles tendon swelling or tenderness




Absent Trendelenberg test
Negative Modified Schobers test: More than 15 cms

Other systems:

Respiratory System: No abnormality detected
Cardiovascular System: No abnormality detected
Abdomen: No abnormalities detected
Nervous System: No abnormalities detected

Provisional Diagnosis:

Multiple symmetrical polyarthritis with chronic duration of around 7 years and with signs of inflammation, involving PIP joints and MCP joints with sparing of DIP joints - Rheumatoid Arthitis
with no other system involvement. 


Investigations:
Metabolic Profile - Normal
ESR: 20 mm/hr
CRP: Positive
RA factor: Positive




Diagnosis:
Multiple symmetrical joint involvement, chronic duration of more than 7 years-  Rheumatoid arthritis
with an ACR - EULAR Criteria of 9 
More than 10 joints involvement - 5, Serology - Score 2, Abnormal CRP - 1, More than 7 years of duration - 1 


Discussion:


Darlington et al. used elimination and oral food challenge to identify foods capable of inducing symptoms in RA patients. Forty-eight patients undertook a 6-week elimination diet; forty-one were found to have foods that triggered symptoms. 



Microbiome and Inflammation

The gut may play a key role in the pathophysiology of RA. Permeability of the intestinal barrier allows for food components or bacterial endotoxins to enter the bloodstream. Absorption of endotoxins into circulation can trigger a systemic inflammatory response.

Kim et al. observed that a vegan diet lowers the relative abundance of Enterobacteriaceae in the gut, which in turn reduces fecal lipocalin-2 (Lcn-2), a sensitive biomarker of intestinal inflammation, within 28 day




Pathogenesis of Rheumatoid Arthritis


Critical appraisal 

Efficacy and safety of hydroxychloroquine sulphate in rheumatoid arthritis: a randomized, double-blind, placebo controlled clinical trial--an Indian experience


P - One hundred and twenty-two patients with RA were enrolled 
I - 61 patients were randomized to receive either hydroxychloroquine tablets, two tablets of 200 mg daily 
C -  61 patients received placebo two tablets daily. 

After 8 weeks all patients received one tablet of hydroxychloroquine 200 mg daily for 4 weeks. Every patient also received one tablet of Nimesulide 100 mg twice daily.
O - 40.4% of patients on hydroxychloroquine showed improvement by modified ACR response criteria whereas only 20.7% ( p = 0.02) showed improvement in the placebo group. No significant side effects were observed in any of the patients. There were no ocular toxicities.



Obesity is implicated in rheumatoid arthritis (RA) development, severity, outcomes, and treatment response. 

The independent effects of overweight and obesity on ability to achieve sustained remission (sREM) in the 3 years following RA diagnosis.

P - 982 patients
315 (32%) had a healthy BMI, 343 (35%) were overweight, and 324 (33%) were obese; 355 (36%) achieved sREM within 3 years. 

O - Compared to healthy BMI, overweight patients and obese patients were significantly less likely to achieve sREM.

Rates of overweight and obesity were high (69%) in this early RA cohort. Overweight patients were 25% less likely, and obese patients were 47% less likely, to achieve sREM in the first 3 years, despite similar initial disease-modifying antirheumatic drug treatment and subsequent biologic use. 


Saturday, May 28, 2022

22 year old woman with Generalized Anasarca and Facial Puffiness

A 22 year old woman presented to our outpatient unit with the complaints of 

Bilateral lower limb swelling since 1 month

Facial puffiness since 1 month 

Shortness of breath since 1 month 

Reduced urine output since 1 week


History  of present illness :

1 month back, she first noticed bilateral lower limb swelling, pitting type upto her ankles which gradually extended to her thighs. Following this she also developed facial puffiness, significantly around her eyes which alarmed her  and she paid a visit to our hospital and was started on conservative medications. However her symptoms did not subside. She started experiencing dyspnea on exertion which gradually progressed to an extent that she found it difficult to even walk for a short distance. She reported frothing of urine, however she had no hematuria. She complaints of reduced urine output since 1 week. 

She did not have any history of chest pain, palpitations, giddiness, sweating. 

She denied any history of fever, sore throat, rash, joint pains. No history of drug intake or antibiotic usage. 

Past History:

12 years back, she was diagnosed with Type 1 Diabetes Mellitus on visiting a local hospital for fever and body pains. She has been receiving insulin injections since then. 

In 2018, she was admitted in a local hospital for slurred speech which eventually became normal on normalising her blood sugar levels. 

Her father reported that she had to be readmitted 2-3 times there after with the same complaints of slurred speech and hyperglycaemia. She was started on Injection Human Insulin Mixtard 18 units in the morning and 12 units at night. 

6 months back, she was diagnosed with Hypertension on routine follow up and was started on Tab Telma 40mg once daily. 

4 months back, she had an abortion at 7 weeks of gestation since the fetal heart beat couldn’t be assessed. 

Personal History: 

She currently lives with her parents who work as daily wage laborers. She has 2 elder sisters, who are married. She studied till 10th standard. She was married 1 year back to a farmer, however they haven’t been living together ever since her miscarriage.

Her mother reported decreased appetite since the last 2 months. She has only been consuming only milk, once to twice a day along with chapatti once in a day. 


Problem Representation:

1. Generalized Anasarca 

2. Dyspnea on exertion 

3. Reduced urine output

4. Uncontrolled blood sugars

Provisional Diagnosis:

1. A 22 year old woman with significicant generalized anasarca,  proteinruria and hypertension - Nephrotic Syndrome - ? Diabetic Nephropathy

2. Anemia secondary to ? chronic disease

3. Type 1 Diabetes Mellitus since 12 years

4. Hypertensive since 6 months


Clinical Examination: 

General Examination:

Pulse Rate: 90 beats per minute, normal on volume, no radio-radial or radio-femoral delay

Blood Pressure: 150/70mmhg, right arm, supine posture 

Respiratory Rate: 22 cycles per minute 

Temperature: 98.6 F

Spo2: 98% on Room Air

GRBS: 260mg/dl 


Height: 152.4 cm

Weight: 58 kgs

Body Mass Index: 23.2 kg/m

Waist circumference: 96 cms




Images from previous admission:






Current admission pictures:

Head to toe:

Hair: Normal colour and texture, No areas of hair loss.


Eyes:

Pallor +

Periorbital edema +

Bilateral pterygium +

No cyanosis, icterus







Right IJV central line:






Left thigh hyperpigmented lesion present since her childhood,
? Acquired nevus



Bilateral lower limb edema, comparatively reduced due to Hemodialysis



Systemic Examination:

Abdomen:

Inspection:

Distended
Umbilicus - Slit like
No visible scars, sinuses, pulsations









Palpation:
No tenderness
No guarding or rigidity
No palpable masses
No organomegaly





Percussion:
Shifting dullness couldn't be elicited


Auscultation:

Bowel sounds +



Nervous System Examination: 

Only positive findings mentioned:

Optic Nerve -Fundoscopy
                      Right eye: Nonproliferative Diabetic Retinopathy
                       Left eye:  Proliferative Diabetic Retinopathy




Respiratory System Examination:
Right infrascapular crepts + on auscultation

Cardiovascular System Examination:
S1, S2+


Investigations:


ECG showing 
Heart Rate of 100 beats per minute
Left Axis deviation
Low voltage complexes
Poor R wave progression

Chest Xray PA view:



 Showing:
 Left side breast shadow
Left pleural effusion
Right IJV central line +




Showing normal sized chambers
with an ejection fraction of 60 %
Concentric LVH
Pericardial Effusion +


Fasting Lipid Profile:
Total cholesterol - 247mg/dl
Triglycerides - 262mg/dl
HDL - 48 mg/dl
LDL - 110 mg/dl
VLDL - 52.4 mg/dl

24 hours urinary protein : 2600mg/day

From the day of admission:





Fasting Lipid Profile:
Total cholesterol - 247mg/dl
Triglycerides - 262mg/dl
HDL - 48 mg/dl
LDL - 110 mg/dl
VLDL - 52.4 mg/dl



Diagnosis:
1. Diabetic Nephropathy with eGFR of 17.5 ml/min/1.73 m and creatinine clearance of 24 mL/min
Diabetic Retinopathy - 
2. Anemia secondary to chronic disease
3. Type 1 Diabetes Mellitus since 12 years
4. Hypertension since 6 months


Discussion:




Stages of Diabetic Nephropathy

Earlier identification of GFR decline would allow interventions to decrease the rate of GFR loss and prolong the time to development of end stage renal disease (ESRD) 



https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4138314/#!po=8.46154

Critical Appraisal:

Dual blockade of the renin–angiotensin system in type 1 patients with diabetic nephropathy

P - 21 type 1 patients with Diabetic Nephropathy

DN was diagnosed if the following criteria were fulfilled: persistent albuminuria >300 mg/24 h in two out of three consecutive determinations, presence of diabetic retinopathy, and no clinical or laboratory evidence of other kidney or renal tract disease

I - Each patient received 2 months of treatment with Irbesartan 300 mg o.d. 
C -  2 months of placebo. 
The study medication was added on top of the patient's usual antihypertensive treatment. 
O -





O - Our short‐term study suggest that dual blockade of the RAS offers additional renal and cardiovascular protection in type 1 patients with diabetic nephropathy. 

If you notice from my perspective, there’s not much of a difference between both the groups 
Cons: Also, 2 months assessment of the effect of Irbesartan wouldn’t be sufficient. We need to assess the long term effects.



The Effect of Intensive Treatment of Diabetes on the Development and Progression of Long-Term Complications in Insulin-Dependent Diabetes Mellitus


P - A total of 1441 patients with IDDM -- 726 with no retinopathy at base line (the primary-prevention cohort) and 715 with mild retinopathy (the secondary-intervention cohort) 
I - 1441 were assigned to intensive therapy administered either with an external insulin pump or by three or more daily insulin injections and guided by frequent blood glucose monitoring or to conventional therapy with one or two daily insulin injections. The patients were followed for a mean of 6.5 years, and the appearance and progression of retinopathy and other complications were assessed regularly.


According to this the patients on intensive phase reduced risk of diabetic retinopathy, only 23 patients developed diabetic retinopathy and 91 patients on conventional therapy developed retinopathy.





In the two cohorts combined, intensive therapy reduced the occurrence of microalbuminuria by 39 percent, that of albuminuria by 54 percent, and that of clinical neuropathy by 60 percent.