Table 1 Summary of recommendations

(1) The ESSDAI should be used as a measurement tool for diagnosing and evaluating the activity of systemic manifestations. (Level of Agreement: 88%, Strength of Recommendation: conditional recommendation)

(2) Inflammatory arthralgia and/or non-erosive arthritis are frequent manifestations in pSS that should be classified according to the number and location of joints involved. Cases of suspected arthritis should be confirmed by physical examination and, if necessary, imaging tests. (Level of Agreement: 100%, Strength of Recommendation: Strong)

(3) Respiratory impairment in pSS is frequent, polymorphic, and may be associated with decreased quality of life, development of lymphoma, and worse prognosis. Thus, we recommend that every patient should be accurately assessed for the presence of respiratory signs and symptoms at diagnosis and follow-up visits. For the confirmation of pulmonary involvement, the most frequently used tests are high-resolution chest tomography and the complete pulmonary function test. Level of Agreement: 100%, Strength of Recommendation: Strong

(4) Considering that pulmonary involvement can be asymptomatic, we recommend that all patients be evaluated by these tests at least once during their evolution, especially those with risk factors for lung function impairment, such as the male sex, presence of rheumatoid factor, antinuclear antibodies (ANA), anti-Ro/SSA and anti-La/SSB, hypergammaglobulinemia, lymphopenia, Raynaud's phenomenon, peripheral arthritis, changes in the pulmonary function test (decreased FVC and FEV1), smoking history, advanced age, gastrointestinal involvement, and focal score ≥ 4 on the labial salivary gland biopsy. (Level of Agreement: 100%, Strength of Recommendation: Strong)

(5) For patients with pulmonary involvement, monitoring with high-resolution chest tomography and the complete pulmonary function test is recommended at least every two years (as proposed in ESSDAI) or earlier, if necessary, according to the clinical judgment. (Level of Agreement: 100%, Strength of Recommendation: Strong)

(6) Renal manifestations are underdiagnosed, as they do not often present evident symptoms; thus, an adequate and systematic assessment of renal function is required. When investigating, we must consider the two types of renal impairment: distal tubulointerstitial nephritis (Type I) and proximal tubulointerstitial nephritis (Type II), with or without renal tubular acidosis (RTA); and glomerulonephritis (GN).; (Level of Agreement: 100%, Strength of Recommendation: Strong)

(7) Initial and follow-up evaluations are recommended even in asymptomatic patients, with the measurement of serum creatinine, glomerular filtration rate (GFR), type I urine (pH and density always assessed in fresh morning urine), serum electrolytes (Na, K, Cl), venous blood gases (HCO3, blood pH), and plain abdominal radiography or renal bladder ultrasound. (Level of Agreement: 100%, Strength of Recommendation: Strong)

(8) The diagnosis of tubulointerstitial involvement requires an active search for RTA signs and symptoms: cramps, muscle weakness, hypokalemic periodic paralysis, renal lithiasis, nephrocalcinosis, polyuria, polydipsia, nocturia, nephrogenic diabetes insipidus, bone pain, and pathological fractures secondary to osteomalacia. (Additional file 1: Chart S2) In these cases, in addition to the initial laboratory evaluation, serum calcium and phosphorus, 24-h proteinuria, or the protein creatinine index (PCI) should be assessed. Hypocitraturia is a frequent and early finding in distal tubular dysfunction, being a risk factor for urolithiasis and nephrocalcinosis (Level of Agreement: 100%, Strength of Recommendation: Strong)

(9) Distal renal tubular acidosis (dRTA) is secondary to tubulointerstitial nephritis (TIN) when urinary pH > 5.5 in the presence of metabolic acidosis, with normal blood anion gap and positive urinary anion gap. If urinary pH > 5.5 in the absence of metabolic acidosis, incomplete distal renal tubular acidosis (idRTA) should be considered. Urinary acidification tests with ammonium chloride or furosemide and hydrocortisone confirm the diagnosis if urinary pH remains > 5.5. If these tests are not available, the patient should be monitored more frequently. In cases of urinary pH > 7.5, proximal renal tubular acidosis (pRTA) should be suspected, which may course with normal glycosuria and glycemia, hyperphosphaturia, hyperuricosuria, aminoaciduria, hypophosphatemia, and hypouricemia. Assessment of these tests should be required. (Level of Agreement: 100%, Strength of Recommendation: Strong)

(10) Glomerular involvement is much less frequent, but presents evident symptoms in most cases and may be associated with cryoglobulinemia. (Additional file 1: Chart S2) Laboratory findings are suggestive of impaired kidney function, proteinuria, hematuria, leukocyturia, cylindruria, and hypocomplementemia. Assessment of this test should be required. (Level of Agreement: 100%, Strength of Recommendation: Strong)

(11) Renal biopsy is indicated for suspected glomerulopathies, cases of tubulointerstitial nephritis with kidney failure or severe electrolyte imbalance, and differential diagnosis. (Level of Agreement: 100%, Strength of Recommendation: Strong)