Clinicopathological Characteristics and Prognosis of Pulmonary Renal Syndrome in Coastal South India

 

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Clinicopathological Characteristics and Prognosis of Pulmonary Renal Syndrome in Coastal South India

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Praveen
Namboodiri
Praveen Namboodiri praveen_namboodiri@yahoo.com Bishop Benziger Hospital Kollam H.O.D Nephrology Kollam India *
Jerin Dsilva Jeenojames@gmail.com Bishop Benziger Hospital Kollam Pulmonary Medicine and Critical Care Kollam India -
Husamath Parayi husamath@gmail.com Daru Shifa Hospital, Kunnumpuram M.D General Medicine Kozikhode India -
Moncy Thomas moncythomas.mt@gmail.com SKF Kollam Chief Dialysis Unit Kollam India -
Rohit Gundeti rohitpgundetiofficial@gmail.com IIT Hyderabad AI and Data science Hyderabad India -
 
 
 
 
 
 
 
 
 
 

To Analyse clinicopathological characteristics, prognosis, and response to standard therapy in Patients with PRS in coastal South India.

Retrospective data of 160 PRS patients were analysed, including demographic, clinical, serological, radiological, BAL, renal histopathology, treatment and outcome parameters.
Standard criteria of protein ≥1 g/day, hematuria >5 RBC/HPF, RBC casts >1/slide, C-ANCA >3 U/mL, P-ANCA >5 U/mL, Anti-GBM >10 U/mL and Anti-dsDNA >25 IU/mL were applied. HRCT findings: ground-glass opacities (GGO) or alveolar infiltrates (AI); BAL findings included: diffuse alveolar haemorrhage (DAH) or interstitial infiltrates (ILI). Histology: diffuse proliferative GN (DPGN), pauci-immune GN (PGN), and acute tubulointerstitial nephritis (ATIN).
Three-year renal survival groups: good recovery (Cr <3 mg%), partial recovery (Cr 3–6 mg%), or CKD 5 (dialysis/transplant). All patients received steroids. Therapy was classified as Cyclophosphamide (CPM) alone, Plasmapheresis (PLX) alone, Rituximab (RTX) alone, CPM + PLX, and RTX + PLX.
Multivariate logistic regression and AI-based models were used to identify predictors of dialysis, ventilation, and renal survival.

1. Demography: PRS was common in the <50 years age group with a clear male preponderance. Figure 1

Figure 1. Age and gender distribution with survival overlay.

2. Clinical Presentation: Figure 2

Figure 2. Frequency of presenting features proteinuria, hematuria, and pulmonary symptoms.

3. Radiological Findings (HRCT): Table 1

Table 1. Radiological patterns on HRCT.

4. BAL Findings: DAH (52%) and ILI (48%) were observed. Both had similar renal survival at 3 years.

5. Histopathology: DPGN was predominant (66%), ATIN (18%) and Pauci-immune CGN (14%). Higher crescent proportion (>50%) was associated with poorer 3-year renal survival (OR 1.43, 95% CI 0.91–2.23; p=0.12).

6. Aetiology: ANCA-associated vasculitis (AAV) was the commonest cause (72%), anti-GBM (18%), and SLE (6%).

7. Treatment Outcomes: No treatment arm showed statistically significant outcomes (p > 0.05). Descriptive trends favoured CPM + PLX and RTX + PLX over monotherapy. Regression showed OR 0.33 (95% CI 0.08–1.38) for CPM + PLX and 0.37 (95% CI 0.11–1.29) for RTX + PLX. Table 2

Table 2. Three-year renal survival and mortality across treatment arms. Descriptive trends favored CPM + PLX and RTX + PLX combinations.

8. Prognostic Indicators: None of the predictors reached statistical significance (p < 0.05), though crescents >50%, dialysis, and ventilation requirement showed clinically relevant trends toward poorer outcomes. Table 3

Table 3. Prognostic factors for 3-year renal survival.

9. Combined Variable Analysis: Table 4

Table 4. Joint significance of variable collections 3-year renal survival.

10. Logistic Regression: 3-Year Renal Survival by Treatment Arm. Table 5

Table 5. Logistic regression: 3-year renal survival by treatment arm.

11. Logistic Regression: Mortality by Treatment Arm. Table 6

Abbreviations: PRS – Pulmonary–Renal Syndrome; GN – Glomerulonephritis; DPGN – Diffuse Proliferative GN; CGN – Crescentic GN; ATIN – Acute Tubulointerstitial Nephritis; HRCT – High-Resolution Computed Tomography; BAL – Bronchoalveolar Lavage; GGO – Ground-Glass Opacities; AI – Alveolar Infiltrates; DAH – Diffuse Alveolar Haemorrhage; ILI – Interstitial Infiltrates; AAV – ANCA-Associated Vasculitis; GBM – Glomerular Basement Membrane; Cr – Serum Creatinine; CPM – Cyclophosphamide; RTX – Rituximab; PLX – Plasmapheresis, NS Not Significant (p > 0.05).

In this coastal South Indian PRS cohort, AAV was the commonest cause with the highest incidence in males under 50 years. Presence of >50% crescents, need for dialysis, and need for mechanical ventilation correlated with lower survival. Prognosis was similar across histological subtypes on kidney biopsy and BAL findings, highlighting their diagnostic rather than prognostic value. Plasmapheresis improved renal survival modestly. Treatment with Rituximab was not superior to cyclophosphamide, though both improved outcomes when used along with plasmapheresis. Overall survival correlated best with initial organ damage, underscoring the importance of early diagnosis and treatment.

Kewords